LEUKEMIA

Leukemia is a type of cancer of the blood or bone marrow characterized by an abnormal increase of immature white blood
cells called "blasts".
Clinically and pathologically, leukemia is subdivided into a variety of large groups. The first division is between
its acuteand chronic forms:
 Acute leukemia is characterized by a rapid increase in the number of immature blood cells. Crowding due to such cells
makes the bone marrow unable to produce healthy blood cells. Immediate treatment is required in acute leukemia due to the
rapid progression and accumulation of the malignant cells, which then spill over into the bloodstream and spread to other
organs of the body. Acute forms of leukemia are the most common forms of leukemia in children.
 Chronic leukemia is characterized by the excessive build up of relatively mature, but still abnormal, white blood cells.
Typically taking months or years to progress, the cells are produced at a much higher rate than normal, resulting in many
abnormal white blood cells. Whereas acute leukemia must be treated immediately, chronic forms are sometimes monitored
for some time before treatment to ensure maximum effectiveness of therapy. Chronic leukemia mostly occurs in older
people, but can theoretically occur in any age group.
Additionally, the diseases are subdivided according to which kind of blood cell is affected. This split divides leukemias into
lymphoblastic or lymphocytic leukemias and myeloid or myelogenous leukemias:
 In lymphoblastic or lymphocytic leukemias, the cancerous change takes place in a type of marrow cell that normally
goes on to form lymphocytes, which are infection-fighting immune system cells. Most lymphocytic leukemias involve a
specific subtype of lymphocyte, the B cell.
 In myeloid or myelogenous leukemias, the cancerous change takes place in a type of marrow cell that normally goes on
to formred blood cells, some other types of white cells, and platelets.
Combining these two classifications provides a total of four main categories. Within each of these four main categories, there are
typically several subcategories. Finally, some rarer types are usually considered to be outside of this classification scheme.
 Acute lymphoblastic leukemia (ALL) is the most common type of leukemia in young children. This disease also affects
adults, especially those age 65 and older. Standard treatments involve chemotherapy and radiotherapy. The survival rates
vary by age: 85% in children and 50% in adults.[4] Subtypes include precursor B acute lymphoblastic leukemia, precursor
T acute lymphoblastic leukemia, Burkitt's leukemia, and acute biphenotypic leukemia.
 Chronic lymphocytic leukemia (CLL) most often affects adults over the age of 55. It sometimes occurs in younger
adults, but it almost never affects children. Two-thirds of affected people are men. The five-year survival rate is 75%.[5] It
is incurable, but there are many effective treatments. One subtype is B-cell prolymphocytic leukemia, a more aggressive
disease.
 Acute myelogenous leukemia (AML) occurs more commonly in adults than in children, and more commonly in men
than women. AML is treated with chemotherapy. The five-year survival rate is 40%, except for APL, which is over 90%.
[6]
 Subtypes of AML include acute promyelocytic leukemia, acute myeloblastic leukemia, and acute megakaryoblastic
leukemia.
 Chronic myelogenous leukemia (CML) occurs mainly in adults; a very small number of children also develop this
disease. Treatment is with imatinib (Gleevec in United States, Glivec in Europe) or other drugs.[7] The five-year survival
rate is 90%.[8][9] One subtype is chronic myelomonocytic leukemia.
 Hairy cell leukemia (HCL) is sometimes considered a subset of chronic lymphocytic leukemia, but does not fit neatly
into this pattern. About 80% of affected people are adult men. No cases in children have been reported. HCL is incurable,
but easily treatable. Survival is 96% to 100% at ten years.[10]
 T-cell prolymphocytic leukemia (T-PLL) is a very rare and aggressive leukemia affecting adults; somewhat more men
than women are diagnosed with this disease.[11] Despite its overall rarity, it is also the most common type of mature T
cell leukemia;[12]nearly all other leukemias involve B cells. It is difficult to treat, and the median survival is measured in
months.
 Large granular lymphocytic leukemia may involve either T-cells or NK cells; like hairy cell leukemia, which involves
solely B cells, it is a rare and indolent (not aggressive) leukemia.[13]
 Adult T-cell leukemia is caused by human T-lymphotropic virus (HTLV), a virus similar to HIV. Like HIV, HTLV
infects CD4+ T-cells and replicates within them; however, unlike HIV, it does not destroy them. Instead, HTLV
"immortalizes" the infected T-cells, giving them the ability to proliferate abnormally. Human T cell lymphotropic virus
types I and II (HTLV-I/II) are endemic in certain areas of the world.

CAUSES

Experts say that different leukemias have different causes. The following are either known causes, or strongly suspected causes:
 Artificial ionizing radiation
 Viruses - HTLV-1 (human T-lymphotropic virus) and HIV (human immunodeficiency virus)
 Benzene and some petrochemicals
 Alkylating chemotherapy agents used in previous cancers
 Maternal fetal transmission (rare)
 Hair dyes
 Genetic predisposition - some studies researching family history and looking at twins have indicated that some people
have a higher risk of developing leukemia because of a single gene or multiple genes.
 Down syndrome - people with Down syndrome have a significantly higher risk of developing leukemia, compared to
people who do not have Down syndrome. Experts say that because of this, people with certain chromosomal abnormalities
may have a higher risk.
 Electromagnetic energy - studies indicate there is not enough evidence to show that ELF magnetic (not electric) fields
that exist currently might cause leukemia. The IARC (International Agency for Research on Cancer) says that studies which
indicate there is a risk tend to be biased and unreliable.
 previous chemotherapy or radiation therapy
 exposure to high doses of radiation or to benzene (found in unleaded gasoline, tobacco smoke, chemical production
facilities)
 family history
 genetic abnormality, such as an abnormality on chromosome 22 (also known as the Philadelphia chromosome)
 All forms of cancer that can spread within the body (malignant), including leukemia, are thought to be due to genetic
abnormalities (mutations). In leukemia, the damage occurs in the bone marrow stem cells. These special cells help to
manufacture all the other cells in the blood. With this condition the production of these cells is out of control.
Unfortunately the specific cause of leukemia is unknown.� There are several suspected factors that may cause
leukemia, but even these factors are not absolute indicators of developing leukemia.� Even though a person may have been
exposed to one or more of the following risk factors, it does not necessarily mean they will develop leukemia.�� In fact, most
people who develop the disease have not been exposed to any risk factors at all�the direct cause of leukemia is still unknown.
 
Suspected Environmental Risks: 
 
�Smoking--about 20 percent of adult acute leukemia cases are related to smoking.
�High doses of radiation, produced by an atomic bomb or a nuclear reactor
accident.
�Long-term exposure to benzene, found in gasoline, which causes a twenty times higher risk of developing acute
mylogenous leukemia.
�Exposure to electromagnetic fields (a type of low-energy radiation that comes from power lines and electric appliances).
Treatment for other types of cancer by a combination of certain chemotherapy drugs and radiation therapy.
Parental exposure to radiation before conception or during early fetal development.
 
 Genetic Risks:
� Myelodysplstidic syndrome--a pre-leukemia condition.
� Chromosome damage�rare genetic syndromes put people at a higher risk.
� Immune system deficiencies--People are more at risk when they have a decreased ability to resist foreign cells.
� Downs syndrome--children born with Downs are twenty times more likely to develop acute leukemia.
� Chronic Myelogenous leukemia--a chromosomal disorder. �The Philadelphia Chromosome hooks up to a different
chromosome than it is supposed to, making the body unable to know when to stop producing white blood cells.
 Viral risks:
� Viruses may cause leukemia in animals, but in humans this only occurs with rare types of leukemia�very uncommon.
� Common forms of leukemia are not contagious.
PATHOPHYSIOLOGY
Leukemia is malignant neoplasms of the cells derived from either the myeloid or lymphoid line of the hematopoietic
stem cells in the bone marrow. Proliferating abnormal and immature cells (blast) spill out into the blood and infiltrate the
spleen, lymph nodes, and other tissue. Acute leukemias are characterized by rapid progression of symptoms. High numbers
(greater than 50,000/mm3) of circulating blast weaken blood vessel walls, with high risk for rupture and bleeding, including
intracranial hemorrhage.
Lymphocytic leukemias involve immature lymphocytes and their progenitors. They arise in the bone marrows but
infiltrate the spleen, lymph nodes, central nervous system (CNS), and other tissues. Myelogenous leukemias involve the
pluripotent myeloid stem cells and, thus, interfere with the maturation of granulocytes, erythrocytes, and thrombocytes.
Acute myelogenous leukemias (AML) and acute lymphatic leukemia (ALL) have similar presentations and courses.
Approximately half of new leukemias are acute. Approximately 85 % of acute leukemias in adults are AML, and incidence
of AML increases with age. ALL is the most common cancer in children, with peak incidence between ages 2 and 9.
Although the cause of leukemias is unknown, predisposing factors include genetic susceptibility, exposure to ionizing
radiation or certain chemicals and toxins, some genetic disorder (Down syndromes, Fanconi’s anemia), and human T-cell
 leukemia-lymphoma virus. Complications include infection, leukostasis leading to hemorrhage, renal failure, tumor lysis
syndrome, and disseminating intravascular coagulation.
SIGNS AND SYMPTOMS

Signs and symptoms of leukemia

A sign is something that can be observed and recognized by a doctor or healthcare professional (for example, a rash).
A symptom is something that only the person experiencing it can feel and know (for example, pain or tiredness). The signs and
symptoms of leukemia can also be caused by other health conditions. It is important to have any unusual symptoms checked by a
doctor.
 
Signs and symptoms of acute leukemia (fast growing) may be similar to the flu and come on suddenly within days or weeks.
 
In the early stages of chronic leukemia (slow growing), many people have few or no symptoms. Signs and symptoms usually
develop gradually and people will complain that they just do not feel well. The disease is often discovered during a routine blood
test.
 
Signs and symptoms of leukemia are:
 fatigue
 malaise
 loss of appetite
 weight loss
 fever
 anemia
o shortness of breath
o paleness
o palpitations (rapid heartbeat)
o weakness
o dizziness
 bleeding
o widespread bruising
o frequent or severe nose bleeds
o bleeding gums
o mid-cycle or heavy menstrual flow
o red spots on the skin (petechiae)
 frequent infections
o lungs (pneumonia)
o urinary tract
o gums
o around the anus
o cold sores
  vomiting
 headache
 sore throat
 night sweats
 bone or joint pain
 enlarged lymph nodes in the neck, underarm, groin or above the collarbone
 abdominal discomfort or feeling of fullness
 vision problems
 sores in the eyes
 swelling of the testicles
Rare signs and symptoms
Rare signs and symptoms of leukemia are:
 chloroma (granulocytic sarcoma) – a tumour-like collection of leukemia cells under the skin or in other parts of the body
occur
 skin changes
o Leukemia cutis occurs when leukemia cells enter the skin. The sores or patches can be any size and are usually
pink or tan in colour.
o Leukocytoclastic vasculitis resembles an allergic reaction on the skin. Sores usually appear on the extremities
(hands and feet).
o Sweet's syndrome (acute febrile neutrophilic dermatosis) includes fever and painful sores that may appear
anywhere on the body.

Diagnosing leukemia
Diagnosis is the process of finding the underlying cause of a health problem. The process of diagnosis may seem long and
frustrating, but it is important for the doctor to rule out other possible reasons for a health problem before making a cancer
diagnosis. Diagnostic tests for leukemia are usually done when:
 the symptoms of leukemia are present
 the doctor suspects leukemia after talking with a person about their health and completing a physical examination
 routine laboratory tests suggest a problem with the blood
 Medical history and physical examination
The medical history is a record of present symptoms, risk factors and all the medical events and problems a person has had in the
past. The medical history of a person's family may also help the doctor to diagnose leukemia.
 
In taking a medical history, the doctor will ask questions about:
 a personal history of
o exposure to high doses of radiation
o genetic syndromes
o exposure to benzene
o previous chemotherapy or radiation therapy
o viral infections
 a family history of leukemia
 signs and symptoms
 
A physical examination allows the doctor to look for any signs of leukemia. During a physical examination, the doctor may:
 measure vital signs for fever, shortness of breath and rapid heartbeat
 assess the skin for bruising and paleness
 feel areas of the neck, underarm and groin for any swollen or enlarged lymph nodes
 examine the mouth for infection and bleeding or swollen gums
 feel the abdomen for enlarged organs
 examine the skeleton for tenderness or pain
Complete blood count
A complete blood count (CBC) measures the number and quality of white blood cells, red blood cells and platelets. Leukemia is
suspected when blood cell counts are abnormal and blood cells do not look normal. Abnormal blood cell counts may be due to
leukemia or other conditions. Blasts (immature white blood cells) are not normally seen in the blood, so leukemia is suspected if
blasts are present.
 acute leukemia:
o White blood cell counts may be low, normal or high.
 Blast cells may be present in the blood of people with acute leukemia.
 Many people with acute leukemia have neutropenia (a low neutrophil count).
o About 40% of people with acute leukemia have thrombocytopenia (a low platelet count).
 o Most people with acute leukemia have anemia (low number of red blood cells).
 chronic leukemia:
o White blood cell count is high.
o Platelet count may be low.
o Anemia may be present.
Blood chemistry tests
Blood chemistry tests measure certain chemicals in the blood. They show how well certain organs are functioning and can also
be used to detect abnormalities. They help to detect problems with the liver or kidney caused by the spread of leukemia cells. The
following blood levels may be elevated:
 blood urea nitrogen (BUN)
 creatinine
 phosphate
 lactate dehydrogenase (LDH)
 alanine aminotransferase (ALT)
 aspartate transaminase (AST)
 uric acid
Bleeding and clotting factors
Tests measure blood clotting factors to see how well the body can clot blood. Abnormal levels of blood clotting factors may
occur with leukemia. They are measured using the following tests:
 fibrinogen level
 prothrombin time (PT) or international normalized ratio (INR)
 partial thromboplastin time (PTT)

Cytochemistry
Cytochemistry uses stains (dyes) to identify tissue structures and cell components in blood or bone marrow cells. Certain stains
are attracted to certain substances found in some types of leukemia cells. The staining results can be seen under a microscope.
Cytochemistry helps determine the type of cells that are present.
 
Flow cytometry
Flow cytometry is a laboratory test that is used to sort, count and examine microscopic particles (such as cells or DNA). Cells are
measured by staining them with a light-sensitive dye, placing them in fluid and passing them through a laser beam. The laser
makes these cells give off a light that is measured and analyzed by a computer. Flow cytometry helps determine the types of cells
that are present.
 
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Immunohistochemistry
Like flow cytometry, immunohistochemistry (or immunocytochemistry) treats a sample of cells from blood or bone marrow with
special antibodies. Instead of using a laser and computer, chemicals are added that make the cell change colour if a certain
antibody attaches to it. The change in colour can only be seen under a microscope. Immunohistochemistry helps determine the
types of cells that are present.
 
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Cytogenetics
Cytogenetic techniques test a sample of blood or bone marrow to help identify segments of individual chromosomes (like bar
codes) and tell them apart. Cytogenetic techniques show chromosomal abnormalities, which help to confirm the diagnosis and
identify the type of leukemia. The results are also helpful in planning treatment and predicting response to treatment. Results for
cytogenetic testing are usually available within 3–4 weeks.
 
Chromosome changes that occur in some people with leukemia include:
 translocations – part of a chromosome attaches to part of a different chromosome
 inversions – part of a chromosome breaks off, flips end-to-end, and rejoins the same chromosome
 loss or gain of a chromosome number
Fluorescent in situ hybridization (FISH)
Fluorescent in situ hybridization (FISH) is similar to cytogenetic testing and can be used to look for specific changes in
chromosomes in blood or bone marrow cells. Some abnormalities that are too small to be found with standard cytogenetic testing
can be found using FISH.
 
FISH uses special dyes that attach only to specific parts of certain chromosomes. It is very accurate and results are usually
available within a couple of days.
Polymerase chain reaction
Polymerase chain reaction (PCR)and reverse transcriptase polymerase chain reaction (RT-PCR) are sensitive genetic tests used to
find specific abnormalities in blood or bone marrow cells. Abnormalities can be found even if very few leukemia cells are
present in a tissue sample.
 
Bone marrow aspiration and biopsy
During a bone marrow aspiration and biopsy, cells are removed from the bone marrow so they can be tested in the laboratory.
The pathology report from the laboratory will confirm whether or not the person has leukemia and, if so, what type of leukemia.
Lumbar puncture
A lumbar puncture (LP) removes a small amount of cerebrospinal fluid (CSF) from the space around the spine for examination
under a microscope. CSF is the fluid that surrounds the brain and spinal cord. A lumbar puncture is done to see if cancer has
spread to the spinal fluid.
Lymph node biopsy
A lymph node biopsy is a type of surgical biopsy. It is called an excisional biopsy because the lymph node is completely
removed. The lymph node is then examined under a microscope to identify the type of cells and the pattern in which they are
growing.
 Chest x-ray
An x-ray uses small doses of radiation to make an image of the body's structures on film. A chest x-ray is used to look for:
 enlarged mediastinal lymph nodes (lymph nodes in the centre of the chest)
 enlarged thymus gland
 buildup of fluid around the lungs
 pneumonia (lung infection)
Computed tomography (CT) scan
A CT scan uses special x-ray equipment to make 3-dimensional and cross-sectional images of organs, tissues, bones and blood
vessels inside the body. A computer turns the images into detailed pictures. It may be used to show enlarged lymph nodes around
the heart, near the trachea (windpipe) or in the back of the abdomen.
Magnetic resonance imaging (MRI)
MRI uses powerful magnetic forces and radio-frequency waves to make cross-sectional images of organs, tissues, bones and
blood vessels. A computer turns the images into 3-dimensional pictures. It is helpful for looking at the brain and spinal cord. It is
most often used when there is concern that the leukemia has spread to the brain.
Ultrasound
Ultrasound uses high-frequency sound waves to make images of structures in the body. It is used to see if internal organs, such as
the kidneys, liver, or spleen, have been affected by leukemia.

TREATMENT OF ACUTE LYMPHOCYTIC LEUKEMIA

Treatment for acute lymphocytic leukemia (ALL) is given by cancer specialists (oncologists). Some specialize in surgery, some
in radiation therapy and others in chemotherapy (drugs). These doctors work with the person with cancer to decide on a treatment
plan.
 
The main treatment for ALL involves the long-term use of chemotherapy and total treatment usually takes about 2 years. An
important part of treatment is central nervous system (CNS) prophylaxis, which is treatment to make sure the leukemia does not
spread to or remain in the brain or spinal fluid.
 
Treatment plans are designed to meet the unique needs of each person with cancer. Treatment decisions for ALL are based on:
 age
 chromosomal abnormalities
 the subtype of ALL
 the phase of ALL
 the person's overall health
Response to treatment
 A complete remission (complete response) is defined as:
o Peripheral blood cell counts return to normal levels.
o Less than 5% of cells in the bone marrow are blasts.
 Minimal residual disease means that:
o Standard tests, such as looking at cells under a microscope, can't find leukemia cells in the bone marrow.
o More sensitive tests, such as flow cytometry or polymerase chain reaction, find evidence that there are still
leukemia cells in the bone marrow.
 Active disease means that the leukemia is still present during treatment or has relapsed after treatment.
 Relapse means that more than 5% of cells in the bone marrow are blasts.
TREATMENT OPTIONS FOR ACUTE LYMPHOCYTIC LEUKEMIA
 chemotherapy
 o used during all phases of ALL treatment and for central nervous system prophylaxis and treatment
o drugs used to treat ALL include:
 vincristine (Oncovin)
 daunorubicin (Cerubidine)
 doxorubicin (Adriamycin)
 idarubicin (Idamycin)
 mitoxantrone (Novantrone)
 cytarabine (Cytosar, Ara-C)
 asparaginase (Kidrolase)
 etoposide (Vepesid, VP-16)
 teniposide (Vumon, VM-26)
 mercaptopurine (Purinethol, 6-MP)
 methotrexate (Methotrexate)
 cyclophosphamide (Cytoxan, Procytox)
 steroids, such as prednisone (Deltasone) or dexamethasone (Decadron, Dexasone)
 radiation therapy
o to prevent spread of ALL to the central nervous system (CNS) or to treat disease that has spread to the CNS
o for total body irradiation before a stem cell transplant
o to relieve bone pain
o to treat the spread of leukemia to areas like the testicles or skin
o sometimes used to shrink a tumour if it is pressing on the trachea
 stem cell transplant
o may be considered for people with ALL during early first remission or following relapse if a partial or complete
remission can be achieved
o a donor lymphocyte infusion (DLI) may be given to boost the immune system for relapse following a stem cell
transplant
 targeted therapy
o people who have the Philadelphia chromosome (Ph+) may be given imatinib (Gleevec) or another tyrosine
kinase inhibitor as part of the chemotherapy regimen
 supportive therapy to manage complications from treatment
o antibiotics and antifungals – drugs to fight infections
o blood products – replacement therapy when blood cell counts are low
o growth factors – to stimulate white blood cell production
o drugs to reduce high levels of some chemicals in the blood that result from rapid cell death at the beginning of
treatment
o leukapheresis – to remove large numbers of white blood cells from the blood
 follow-up after treatment is finished
o It is important to have frequent follow-up visits, especially in the first 5 years after treatment, even if there are no
signs of disease.
o The time between examinations will become longer as time goes on, but they will continue indefinitely.

TREATMENT OF ACUTE MYELOGENOUS LEUKEMIA

Treatment for acute myelogenous leukemia (AML) is given by cancer specialists (oncologists or hematologists). Some specialize
in surgery, some in radiation therapy and others in chemotherapy (drugs). These doctors work with the person with cancer to
decide on a treatment plan.
 
Treatment plans are designed to meet the unique needs of each person with cancer. Treatment decisions for AML are based on:
 the person's age
 the subtype of AML
 chromosomal (genetic) abnormalities
 whether the person has had chemotherapy before to treat a different cancer
 whether the person has had myelodysplastic syndrome (MDS)
 whether the cancer has spread to the central nervous system (CNS)
 the person's overall health
Response to treatment
 A complete remission (complete response, CR) is defined as the return of peripheral blood cell counts to normal levels
and less than 5% of cells in the bone marrow are blasts.
 Minimal residual disease means that:
o Standard tests, such as looking at cells under a microscope, can't find leukemia cells in the bone marrow.
 o More sensitive tests, such as flow cytometry or polymerase chain reaction, find evidence that there are still
leukemia cells in the bone marrow.
 Active disease means either the leukemia is still present during treatment or has relapsed after treatment. Relapse is
defined as more than 5% of cells in the bone marrow are blasts.
TREATMENT OPTIONS FOR ACUTE MYELOGENOUS LEUKEMIA
 Chemotherapy
o used during all phases of AML treatment and for central nervous system treatment and prophylaxis
o drugs used to treat AML include:
 cytarabine (Cytosar, Ara-C)
 daunorubicin (Cerubidine)
 doxorubicin (Adriamycin)
 idarubicin (Idamycin)
 mitoxantrone (Novantrone)
 thioguanine (Lanvis, 6-TG)
 mercaptopurine (Purinethol, 6-MP)
 fludarabine (Fludara)
 etoposide (Vepesid, VP-16)
 cyclophosphamide (Cytoxan, Procytox)
 topotecan (Hycamtin)
 methotrexate (Methotrexate)
 hydroxyurea (Hydrea)
 amsacrine (AMSA P-D)
 azacitidine (Vidaza)
o drugs used to treat acute promyelocytic leukemia (APL) include:
 tretinoin (all-trans retinoic acid, ATRA, Vesanoid)
 arsenic trioxide
 stem cell transplant
o may be considered for people with AML as consolidation therapy, or if a second remission is achieved after an
early relapse
o a donor lymphocyte infusion (DLI) may be given to boost the immune system for relapse following a stem cell
transplant
 radiation therapy
o to treat disease that has spread to the brain
o to treat a large, localized buildup of leukemia cells outside the bone marrow
o for total body irradiation before a stem cell transplant
o to relieve pain in an area of bone
 supportive therapy to manage complications of treatment
o antibiotics and antifungals – drugs to fight infections
o blood products – replacement therapy when blood cell counts are low
o growth factors – to stimulate white blood cell production
o drugs to reduce high levels of some chemicals in the blood that result from rapid cell death at the beginning of
treatment
o leukapheresis – to remove large numbers of white blood cells from the blood
 follow-up after treatment is finished
o It is important to have frequent follow-up visits for several years after treatment is finished, even if there are no
signs of disease.
o The time between examinations will become longer as time goes on, but they will continue indefinitely.

TREATMENT OF CHRONIC LYMPHOCYTIC LEUKEMIA

Treatment for chronic lymphocytic leukemia (CLL) is given by cancer specialists (oncologists). Some specialize in surgery, some
in radiation therapy and others in chemotherapy (drugs). These doctors work with the person with cancer to decide on a treatment
plan.
 
Treatment plans are designed to meet the unique needs of each person with cancer. At the time of diagnosis, most people with
CLL do not need treatment. Treatment usually begins when the symptoms develop or if the disease starts to progress rapidly.
TREATMENT OPTIONS FOR CHRONIC LYMPHOCYTIC LEUKEMIA
 watchful waiting (watching for disease progression without giving any treatment)
o may be an option if there is evidence of CLL but the person is not experiencing symptoms
  chemotherapy
o as the primary treatment to return blood cell counts to normal and control them
o to reduce an enlarged spleen
o for relapsed or refractory CLL
o chemotherapy drugs used in treating CLL include:
 fludarabine (Fludara)
 cladribine (2-CDA, Leustatin)
 chlorambucil (Leukeran)
 cyclophosphamide (Cytoxan, Procytox)
 vincristine (Oncovin)
 doxorubicin (Adriamycin)
o a steroid, such as prednisone (Deltasone), may be used in combination with chemotherapy
 biological therapy
o for intermediate-risk or high-risk CLL
o when CLL is no longer responding to chemotherapy
o along with chemotherapy as first-line or second-line therapy
o biological therapies used to treat CLL include:
 alemtuzumab (Campath)
 rituximab (Rituxan)
 surgery
o splenectomy (removal of the spleen) may be done
 if the spleen is enlarged and painful, and chemotherapy or radiation therapy does not decrease the
size of the spleen
 to help improve blood cell counts
 radiation therapy
o to reduce the size of the spleen if chemotherapy does not work
o to relieve bone pain caused by the growth of leukemia cells within the bone marrow
o to shrink enlarged lymph nodes in one area of the body
 stem cell transplant
o The timing and use of a stem cell transplant for people with CLL is not standard at this time.
o Researchers continue to evaluate this as a treatment option for CLL.
 supportive therapy to manage complications of treatment
o antibiotics and antifungals – to fight infections
o gamma globulin – to help correct immune deficiency
o blood products – replacement therapy when blood cell counts are low
o growth factors – to stimulate white blood cell production
o drugs to reduce high levels of some chemicals in the blood due to rapid cell death at the beginning of treatment
o leukapheresis – to remove large numbers of white blood cells from the blood
o corticosteroids – to improve red blood cell or platelet counts
 follow-up after treatment is finished
o It is important to have regular follow-up visits for many years after treatment even if there are no signs of
disease.
o The time between examinations will become longer as time goes on, but they will continue indefinitely.
TREATMENT OF CHRONIC MYELOGENOUS LEUKEMIA
Treatment for chronic myelogenous leukemia (CML) is given by cancer specialists (oncologists). Some specialize in surgery,
some in radiation therapy and others in chemotherapy (drugs). These doctors work with the person with cancer to decide on a
treatment plan.
 
Treatment plans are designed to meet the unique needs of each person with cancer. Treatment decisions for CML are based on
the:
 person's age
 phase of CML
 number of blasts in the blood or bone marrow
 size of the spleen
 person's overall health
Response to treatment
Monitoring the blood and bone marrow for response to treatment is an important part of treatment for CML. Blood cell counts
are watched closely and the blood and bone marrow is monitored for presence of the Philadelphia chromosome. If the
Philadelphia chromosome cannot be found, a sensitive test called the polymerase chain reaction (PCR) test may be done to find
small amounts of the BCR-ABL gene (which results from the Philadelphia chromosome).
Disease monitoring
Disease monitoring during treatment is tailored to each person and may include a schedule similar to the following:
 complete blood count (CBC) every week until the counts are stable, and then monthly
 cytogenetic tests (analysis of the cells) of the bone marrow
o at diagnosis
o at 6 and 12 months after treatment starts
o then yearly with continuing treatment
 PCR or reverse transcriptase PCR (RT-PCR) blood tests at diagnosis and approximately every 3 months with continuing
treatment
Hematologic response
The hematologic response usually occurs within the first 3 months of starting treatment.
 complete hematologic response
o The blood cell counts have returned to normal.
o No immature cells are seen in the blood.
o The spleen has returned to normal size.
 partial hematologic response
o This is similar to a complete response, but all 3 conditions have not been achieved.
Cytogenetic response
The cytogenetic response may take several months or longer to occur. A partial cytogenetic response within 3–6 months of
starting treatment predicts an 80–95% chance of achieving a complete cytogenetic response.
 complete cytogenetic response
o No cells with the Philadelphia chromosome are found in the blood or bone marrow.
 partial cytogenetic response
o Less than 35% of cells have the Philadelphia chromosome.
 major cytogenetic response
o This is sometimes used to refer to either a complete or partial response.
 minor cytogenetic response
o 35–90% of cells have the Philadelphia chromosome.
 
There is no specific deadline for a complete cytogenetic response before doctors will consider trying a different treatment. A
different treatment approach is decided on an individual basis. It is based on the person’s age and other possible treatment
options.
Molecular response
Molecular response is based on the polymerase chain reaction (PCR) test results.
 complete molecular response
o The BCR-ABL gene is not found in the blood.
 major molecular response
o A very low amount of BCR-ABL gene is found in the blood.
TREATMENT OPTIONS FOR CHRONIC MYELOGENOUS LEUKEMIA
 targeted therapy – tyrosine kinase inhibitors are the first-line therapy for most people with CML and include:
o imatinib (Gleevec)
o dasatinib (Sprycel)
o nilotinib (Tasigna)
 stem cell transplant
o as a first-line therapy for some people during the chronic phase of the disease
o as a second-line therapy after CML becomes resistant to treatment with imatinib, dasatinib or interferon alfa
o a reduced-intensity transplant may be an option for older people
o a donor lymphocyte infusion (DLI) may be given to boost the immune system when there is relapse following a
stem cell transplant
 biological therapy
o Interferon alfa (Intron A, Roferon A) used to be the first-line therapy for CML. It is still used to treat people who
cannot tolerate or whose CML is resistant to targeted therapy.
 chemotherapy
o Chemotherapy was once one of the main treatments for CML. It may be still used to treat people when targeted
therapy has stopped working, and is part of the treatment during a stem cell transplant.
o Chemotherapy drugs used to treat CML include:
 busulfan (Myleran [oral], Busulfex [intravenous])
  hydroxyurea (Hydrea, Apo-hydroxyurea, Gen-hydroxyurea)
 cytarabine (Cytosar, Ara-C)
 surgery
o Splenectomy (surgical removal of the spleen) may be done if the spleen is enlarged and painful, and
chemotherapy or radiation therapy does not help make the spleen smaller. The spleen may also be removed to
help improve blood cell counts.
 radiation therapy
o to reduce the size of the spleen when chemotherapy is not effective
o to treat disease that may have spread outside the bone marrow
o to relieve bone pain caused by the buildup of leukemia cells in the bone marrow
o for total body irradiation before a stem cell transplant
 supportive therapy to manage complications from treatment
o antibiotics and antifungals – drugs to fight infections
o blood products – replacement therapy when blood cell counts are low
o growth factors – to stimulate white blood cell production
o drugs to reduce high levels of some chemicals in the blood that result from rapid cell death at the beginning of
treatment
o leukapheresis – to remove large numbers of white blood cells from the blood
 follow-up after treatment is finished
o It is important to have regular follow-up visits for many years after treatment, even if there are no signs of
disease.
o The time between examinations will become longer as time goes on, but they will continue indefinitely.
Nursing care
Nursing Priorities
1. Prevent infection during acute phases of disease/treatment.
2. Maintain circulating blood volume.
3. Alleviate pain.
4. Promote optimal physical functioning.
5. Provide psychologic support.
6. Provide information about disease process/prognosis and treatment needs.
Discharge Goals
1. Complications prevented/minimized.
2. Pain relieved/controlled.
3. ADLs met by self or with assistance.
4. Dealing with disease realistically.
5. Disease process/prognosis and therapeutic regimen understood.
6. Plan in place to meet needs after discharge.
Refer to CP: Cancer, for further discussion/expansion of interventions related to cancer care and for client teaching.
NURSING DIAGNOSIS: risk for Infection
Risk factors may include
Inadequate secondary defenses: alterations in mature WBCs (low granulocyte and abnormal lymphocyte count), increased
number of immature lymphocytes; immunosuppression, bone marrow suppression (effects of therapy/transplant)
Inadequate primary defenses (stasis of body fluids, traumatized tissue)
Invasive procedures
Malnutrition; chronic disease
Possibly evidenced by
[Not applicable; presence of signs and symptoms establishes an actual diagnosis.]
DESIRED OUTCOMES/EVALUATION CRITERIA—CLIENT WILL:
Knowledge: Infection Control (NOC)
Identify actions to prevent/reduce risk of infection.
Demonstrate techniques, lifestyle changes to promote safe environment, achieve timely healing.
ACTIONS/INTERVENTIONS RATIONALE
Infection Protection (NIC) 1. Protect client from potential sources of
Independent pathogens/infection.Note: Profound bone marrow
1. Place in private room. Screen/limit visitors as suppression, neutropenia, and chemotherapy place client
indicated. Prohibit use of live plants/cut flowers. at great risk for infection.
Restrict fresh fruits and vegetables or make sure they 2. Prevents cross-contamination/reduces risk of
are washed or peeled. infection.
2. Require good hand-washing protocol for all 3. Although fever may accompany some forms of
personnel and visitors. chemotherapy, progressive hyperthermia occurs in some
3. Monitor temperature. Note correlation
between temperature elevations and chemotherapy
treatments. Observe for fever associated with
tachycardia, hypotension, subtle mental changes.
4. Prevent chilling. Force fluids, administer
tepid sponge bath.
5. Encourage frequent turning and deep
breathing.
6. Auscultate breath sounds, noting crackles,
rhonchi; inspect secretions for changes in
characteristics; e.g., increased sputum production or
change in sputum color. Observe urine for signs of
infection; e.g., cloudy, foul-smelling, or presence of
urgency or burning with voids.
7. Handle client gently. Keep linens
dry/wrinkle free.
8. Inspect skin for tender, erythematous areas;
open wounds. Cleanse skin with antibacterial
solutions.
9. Inspect oral mucous membranes. Provide
good oral hygiene. Use a soft toothbrush, sponge, or
swabs for frequent mouth care.
10. Promote good perianal hygiene. Examine
perianal area at least daily during acute illness.
Provide sitz baths, using Betadine or Hibiclens if
indicated. Avoid rectal temperatures, use of
suppositories.
11. Coordinate procedures and tests to allow for
uninterrupted rest periods.
12. Encourage increased intake of foods high in
protein and fluids with adequate fiber.
1. Avoid/limit invasive procedures (e.g.,
venipuncture and injections) as possible.
Collaborative
1. Monitor laboratory studies, e.g.:
2. CBC, noting whether WBC count falls or
sudden changes occur in neutrophils;
3. Gram’s stain cultures/sensitivity.
4. Review serial chest x-rays.
5. Prepare for/assist with leukemia-specific
treatments such as chemotherapy, radiation, and/or
bone marrow transplant.
6. Administer medications as indicated, e.g.:
antibiotics;
7. Colony-stimulating factors (CSFs): e.g.,
sargramostim (Leukine), filgrastim (Neupogen),
pegfilgrastim (Neulasta).
8. Avoid use of aspirin-containing antipyretics. for client and care providers.
9. Provide nutritious diet, high in protein and 4.
calories, avoiding raw fruits, vegetables, or uncooked infection.
meats.
NURSING DIAGNOSIS: risk for deficient Fluid Volume
Risk factors may include
Excessive losses; e.g., vomiting, hemorrhage, diarrhea
types of infections, and fever (unrelated to drugs or blood
products) occurs in most leukemia
clients. Note:Septicemia may occur without fever.
4. Helps reduce fever, which contributes to fluid
imbalance, discomfort, and CNS complications.
5. Prevents stasis of respiratory secretions, reducing
risk of atelectasis/pneumonia.
6. Early intervention is essential to prevent
sepsis/septicemia in immunosuppressed person.
7. Prevents sheet burn/skin excoriation.
8. May indicate local infection.Note: Open wounds
may not produce pus because of insufficient number of
granulocytes.
9. The oral cavity is an excellent medium for
growth of organisms and is susceptible to ulceration and
bleeding.
10. Promotes cleanliness, reducing risk of perianal
abscess; enhances circulation and healing.Note: Perianal
abscess can contribute to septicemia and death in
immunosupressed clients.
11. Conserves energy for healing, cellular
regeneration.
12. Promotes healing and prevents
dehydration. Note: Constipation potentiates retention of
toxins and risk of rectal irritation/tissue injury.
1. Break in skin could provide an entry for
pathogenic/potentially lethal organisms. Use of central
venous lines (e.g., tunneled catheter or implanted port)
can effectively reduce need for frequent invasive
procedures and risk of infection. Note:Myelosuppression
may be cumulative in nature, especially when multiple
drug therapy (including steroids) is prescribed.
Decreased numbers of normal/mature WBCs can result
from the disease process or chemotherapy, compromising
the immune response and increasing risk of infection.
1. Verifies presence of infections; identifies
specific organisms and appropriate therapy.
2. Indicator of development/resolution of
respiratory complications.
3. Leukemia is usually treated with a combination
of these agents, each requiring specific safety precautions
May be given prophylactically or to treat specific
5. Restores WBCs destroyed by chemotherapy and
reduces risk of severe infection and death in certain types
of leukemia.
6. Aspirin can cause gastric bleeding and further
decrease platelet count.
7. Proper nutrition enhances immune system.
Minimizes potential sources of bacterial contamination.
Decreased fluid intake; e.g., nausea, anorexia
Increased fluid need; e.g., hypermetabolic state, fever, predisposition for kidney stone formation/tumor lysis syndrome
Possibly evidenced by
[Not applicable; presence of signs and symptoms establishes an actual diagnosis.]
DESIRED OUTCOMES/EVALUATION CRITERIA—CLIENT WILL:
Hydration (NOC)
Demonstrate adequate fluid volume, as evidenced by stable vital signs; palpable pulses; urine output, specific gravity, and pH
within normal limits.
Risk Control (NOC)
Identify individual risk factors and appropriate interventions.
Initiate behaviors/lifestyle changes to prevent development of dehydration.
ACTIONS/INTERVENTIONS RATIONALE
Fluid Management (NIC) 1. Tumor lysis syndrome occurs when destroyed
Independent cancer cells release toxic levels of potassium,
1. Monitor I&O. Calculate insensible losses and phosphorus, and uric acid. Elevated phosphorus and
fluid balance. Note decreased urine output in presence uric acid levels can cause crystal formation in the renal
of adequate intake. Measure urine specific gravity and tubules, impairing filtration and leading to renal
pH. failure.
2. Weigh daily. 2. Measure of adequacy of fluid replacement and
3. Monitor BP and HR. kidney function. Continued intake greater than output
4. Evaluate skin turgor, capillary refill, and may indicate renal insult/obstruction.
general condition of mucous membranes. 3. Changes may reflect effects of hypovolemia
5. Note presence of nausea, fever. (bleeding/dehydration).
6. Encourage fluids of up to 3–4 L/day when 4. Indirect indicators of fluid status/hydration.
oral intake is resumed. 5. Affects intake, fluid needs, and route of
replacement.
6. Promotes urine flow, prevents uric acid
precipitation, and enhances clearance of antineoplastic
drugs.
Bleeding Precautions (NIC) 1. Suppression of bone marrow and platelet
1. Inspect skin/mucous membranes for production places client at risk for
petechiae, ecchymotic areas; note bleeding gums, spontaneous/uncontrolled bleeding.
frank or occult blood in stools and urine, oozing from 2. Fragile tissues and altered clotting mechanisms
invasive line sites. increase the risk of hemorrhage following even minor
2. Implement measures to prevent tissue trauma.
injury/bleeding; e.g., gentle brushing of teeth or gums 3. When bleeding is present, even gentle brushing
with soft toothbrush, cotton swab, or sponge-tipped may cause more tissue damage. Alcohol has a drying
applicator; using electric razor instead of sharp razors effect and may be painful to irritated tissues.
when shaving; avoiding forceful nose blowing and 4. May help reduce gum irritation.
needlesticks when possible; using sustained pressure 5. Maintains fluid/electrolyte balance in the
(sandbags or pressure dressings) on oozing absence of oral intake; prevents or minimizes tumor
puncture/IV sites. lysis syndrome, reduces risk of renal complications.
3. Limit oral care to mouth rinse if indicated 6. Relieves nausea/vomiting associated with
(e.g., a mixture of 1/4 tsp baking soda and 1/8 tsp salt administration of chemotherapy agents.
in 8 oz water; may use hydrogen peroxide in water or 7. Improves renal excretion of toxic byproducts
saline for bleeding or infected oral tissue). Avoid from breakdown of leukemia cells. Reduces the
mouthwashes with alcohol. chances of nephropathy as a result of uric acid
4. Provide soft diet. production.
Fluid Management (NIC) 8. May be used to alkalinize the urine, preventing
Collaborative or minimizing tumor lysis syndrome/kidney stones.
1. Administer IV fluids as indicated. 9. Reducing nausea enhances oral intake.
2. Administer medications as indicated, e.g.: 10. When the platelet count is less than 20,000/mm
3. Antiemetics: 5-HT3receptor antagonist drugs (because of proliferation of WBCs and/or bone marrow
such as ondansetron (Zofran) or granisetron (Kytril); suppression secondary to antineoplastic drugs), client
4. Allopurinol (Zyloprim); is prone to spontaneous life-threatening bleeding.
5. Potassium acetate or citrate, sodium Decreasing Hb/Hct is indicative of bleeding (may be
bicarbonate; occult).
6. Antiemetics. 11. Restores/normalizes RBC count and oxygen-
7. Bleeding Precautions (NIC) carrying capacity to correct anemia. Used to
8. Monitor laboratory studies; e.g., platelets, prevent/treat hemorrhage.
Hb/Hct, clotting. 12. Eliminate peripheral venipuncture as source of
9. Administer RBCs, platelets, clotting factors. bleeding.
10. Maintain external central vascular access 13. Helpful in reducing straining at stool which
device (subclavian or tunneled catheter or implanted can cause trauma to rectal tissues.
port). 14. Minimizes blood loss by stopping or slowing
11. Administer medications, e.g.: menstrual flow.
12. Stool softeners;
13. Oral contraceptives.
NURSING DIAGNOSIS: acute Pain
May be related to
Physical agents; e.g., enlarged organs/lymph nodes, bone marrow packed with leukemic cells
Chemical agents; e.g., antileukemic treatments
Psychologic manifestations; e.g., anxiety, fear
Possibly evidenced by
Reports of pain (bone, nerve, headaches, and so forth)
Guarding/distraction behaviors, facial grimacing, alteration in muscle tone
Autonomic responses
DESIRED OUTCOMES/EVALUATION CRITERIA—CLIENT WILL:
Pain Level (NOC)
Report pain is relieved/controlled.
Appear relaxed and able to sleep/rest appropriately
ACTIONS/INTERVENTIONS      RATIONALE
Pain Management (NIC) 1. Helpful in assessing need for intervention; may
Independent indicate developing complications.
1. Investigate reports of pain. Note changes in 2. May be useful in evaluating verbal comments and
degree (use scale of 0–10) and site. effectiveness of interventions.
2. Monitor vital signs, note nonverbal cues; e.g., 3. Promotes rest and enhances coping abilities.
muscle tension, restlessness. 4. May decrease associated bone/joint discomfort.
3. Provide quiet environment and reduce 5. Improves tissue circulation and joint mobility.
stressful stimuli; e.g., noise, lighting, constant 6. Minimizes need for/enhances effects of medication.
interruptions. 7. Successful management of pain requires client
4. Place in position of comfort and support involvement. Use of effective techniques provides positive
joints, extremities with pillows/padding. reinforcement, promotes sense of control, and prepares client
5. Reposition periodically and provide/assist for interventions to be used after discharge.
with gentle ROM exercises. 8. Using own learned perceptions/behaviors to manage
6. Provide comfort measures (e.g., massage, cool pain can help client cope more effectively.
packs) and psychologic support (e.g., encouragement, 9. Facilitates relaxation, augments pharmacologic
presence). therapy, and enhances coping abilities.
7. Review/promote client’s own comfort 10. Helps with pain management by redirecting
interventions; e.g., position, physical attention.
activity/nonactivity. 11. Rapid turnover and destruction of leukemic cells
8. Evaluate and support client’s coping during chemotherapy can elevate uric acid, causing swollen
mechanisms. painful joints in some clients. Note:Massive infiltration of
9. Encourage use of stress management WBCs into joints can also result in intense pain.
   techniques; e.g., relaxation/deep-breathing exercises, 12. Given for mild pain not relieved by comfort
guided imagery, visualization, therapeutic touch. measures. Note: Avoid aspirin-containing products because
1. Assist with/provide diversional activities, they may potentiate hemorrhage.
relaxation techniques. 13. Use around-the-clock, rather than prn, when pain is
Collaborative severe. Note: Use of patient-controlled analgesia (PCA) is
1. Monitor uric acid level as appropriate. beneficial in preventing peaks and valleys associated with
2. Administer medications as indicated: intermittent drug administration and increases client’s sense
3. Analgesics; e.g., acetaminophen (Tylenol); of control.
4. Opioids; e.g., codeine, morphine, 14. May be given to enhance the action of
hydromorphone (Dilaudid); analgesics/opioids.
5. Antianxiety agents; e.g., diazepam (Valium),
lorazepam (Ativan).

NURSING DIAGNOSIS: Activity Intolerance

May be related to
Generalized weakness; reduced energy stores, increased metabolic rate from massive production of leukocytes
Imbalance between oxygen supply and demand (anemia/hypoxia)
Therapeutic restrictions (isolation/bedrest); effect of drug therapy
Possibly evidenced by
Verbal report of fatigue or weakness
Exertional discomfort or dyspnea
Abnormal HR or BP response
DESIRED OUTCOMES/EVALUATION CRITERIA—CLIENT WILL:Endurance (NOC)
Report a measurable increase in activity tolerance.
Participate in ADLs to level of ability.
Demonstrate a decrease in physiological signs of intolerance; e.g., pulse, respiration, and BP remain within client’s normal range.
ACTIONS/INTERVENTIONS RATIONALE
Energy Management (NIC) 1. Effects of leukemia, anemia, and chemotherapy may be
Independent cumulative (especially during acute and active treatment phase),
1. Evaluate reports of fatigue, noting inability to necessitating assistance.
participate in activities or ADLs. 2. Helps client prioritize activities and arrange them around
2. Encourage client to keep a diary of daily fatigue pattern.
routines and energy levels, noting activities that 3. Restores energy needed for activity and cellular
increase fatigue. regeneration/tissue healing.
3. Provide quiet environment and uninterrupted 4. Maximizes available energy for self-care tasks.
rest periods. Encourage rest periods before meals. 5. Smaller meals require less energy for digestion than larger
4. Implement energy-saving techniques; e.g., meals. Increased intake provides fuel for energy. (Refer to CP:
sitting, rather than standing, use of shower chair. Cancer, ND: imbalanced Nutrition: less than body requirements.)
Assist with ambulation/other activities as indicated. 1. Maximizes oxygen available for cellular uptake, improving
5. Recommend small, nutritious, high-protein tolerance of activity.
meals and snacks throughout the day. Schedule meals
around chemotherapy. Give oral hygiene before
meals.
Collaborative
1. Provide supplemental oxygen.
 
NURSING DIAGNOSIS: deficient Knowledge [Learning Need] regarding disease, prognosis, treatment, self-care, and discharge
needs
May be related to
Lack of exposure to resources
Information misinterpretation/lack of recall
Possibly evidenced by
Verbalization of problem/request for information
Statement of misconception
DESIRED OUTCOMES/EVALUATION CRITERIA—CLIENT WILL:
Knowledge: Illness Care (NOC)
Verbalize understanding of condition/disease process and potential complications.
Verbalize understanding of therapeutic needs.
Initiate necessary lifestyle changes.
Participate in treatment regimen.
ACTIONS/INTERVENTIONS RATIONALE
Teaching: Disease Process (NIC) 1. Treatments can include various antineoplastic drugs,
Independent transfusions, peripheral progenitor (stem) cell transplant or bone marrow
1. Review pathology of specific form of transplant.
leukemia and various treatment options.
 
POTENTIAL CONSIDERATIONS following acute hospitalization (dependent on client’s age, physical
condition/presence of complications, personal resources, and life responsibilities)
risk for Infection—inadequate secondary defenses: alterations in mature WBCs (low granulocyte and abnormal lymphocyte
count), increased number of immature lymphocytes, immunosuppression, bone marrow suppression (effects of therapy/
transplant).
ineffective Role Performance—situational crisis, health alterations, change in physical capacity.
ineffective Therapeutic Regimen Management—complexity of therapeutic regimen, decisional conflicts, economic difficulties,
excessive demands made on individual or family, perceived benefits, powerlessness.
interrupted Family Processes—situational crisis (illness, disabling/expensive treatments).

Hemophilia

Hemophilia (heem-o-FILL-ee-ah) is a rare bleeding disorder in which the blood doesn't clot normally.
If you have hemophilia, you may bleed for a longer time than others after an injury. You also may bleed inside your body
(internally), especially in your knees, ankles, and elbows. This bleeding can damage your organs and tissues and may be life
threatening.
OVERVIEW
Hemophilia usually is inherited. "Inherited” means that the disorder is passed from parents to children through genes.
People born with hemophilia have little or no clotting factor. Clotting factor is a protein needed for normal blood clotting. There
are several types of clotting factors. These proteins work with platelets (PLATE-lets) to help the blood clot.
Platelets are small blood cell fragments that form in the bone marrow—a sponge-like tissue in the bones. Platelets play a major
role in blood clotting. When blood vessels are injured, clotting factors help platelets stick together to plug cuts and breaks on the
vessels and stop bleeding.
The two main types of hemophilia are A and B. If you have hemophilia A, you're missing or have low levels of clotting factor
VIII (8). About 8 out of 10 people who have hemophilia have type A. If you have hemophilia B, you're missing or have low
levels of clotting factor IX (9).
Rarely, hemophilia can be acquired. "Acquired” means you aren't born with the disorder, but you develop it during your lifetime.
This can happen if your body forms antibodies (proteins) that attack the clotting factors in your bloodstream. The antibodies can
prevent the clotting factors from working.
This article focuses on inherited hemophilia.
OUTLOOK
Hemophilia can be mild, moderate, or severe, depending on how much clotting factor is in your blood. About 7 out of 10 people
who have hemophilia A have the severe form of the disorder.
People who don't have hemophilia have a factor VIII activity of 100 percent. People who have severe hemophilia A have a factor
VIII activity of less than 
1 percent.
Hemophilia usually occurs in males (with rare exceptions). About 1 in 5,000 males are born with hemophilia each year.
Other Names for Hemophilia
Hemophilia A
 Classic hemophilia
 Factor VIII deficiency
Hemophilia B
 Christmas disease
 Factor IX deficiency
CAUSES
If you have inherited hemophilia, you're born with the disorder. It's caused by a defect in one of the genes that determine how the
body makes blood clotting factor VIII or IX. These genes are located on the X chromosomes (KRO-muh-somz).
Chromosomes come in pairs. Females have two X chromosomes, while males have one X and one Y chromosome. Only the X
chromosome carries the genes related to clotting factors.
A male who has a faulty hemophilia gene on his X chromosome will have hemophilia. A female must have the faulty gene on
both of her X chromosomes to have hemophilia, which is very rare.
If a female has the faulty gene on only one of her X chromosomes, she is a "hemophilia carrier.” Carriers don't have hemophilia,
but they can pass the faulty gene to their children.
Below are two examples of how the hemophilia gene is inherited.
Inheritance Pattern for Hemophilia—Example 1
 The image shows one example of how the hemophilia gene is inherited. In this example, the father doesn't have hemophilia
(that is, he has two normal chromosomes—X and Y). The mother is a carrier of hemophilia (that is, she has one faulty X
chromosome and one normal X chromosome).
Each daughter has a 50 percent chance of inheriting the faulty gene from her mother and being a carrier. Each son has a 50
percent chance of inheriting the faulty gene from his mother and having hemophilia.
Inheritance Pattern for Hemophilia—Example 2

The image shows one example of how the hemophilia gene is inherited. In this example, the father has hemophilia (that
is, his X chromosome is faulty). The mother isn't a hemophilia carrier (that is, she has two normal X chromosomes).
Each daughter will inherit the faulty gene from her father and be a carrier. None of the sons will inherit the faulty gene from
their father; thus, none will have hemophilia.
Females who are hemophilia carriers usually have enough clotting factors from their one normal X chromosome to
prevent serious bleeding problems. However, up to 50 percent of carriers may have an increased risk of bleeding.
Very rarely, a girl is born with hemophilia. This can happen if her father has hemophilia and her mother is a carrier.
Some males who have the disorder are born to mothers who aren't carriers. In these cases, a mutation (random change) occurs in
the gene as it is passed to the child.
What Are the Signs and Symptoms of Hemophilia?
The major signs and symptoms of hemophilia are excessive bleeding and easy bruising.
Excessive Bleeding
The extent of bleeding depends on how severe the hemophilia is.
Children who have mild hemophilia may not have signs unless they have excessive bleeding from a dental procedure, an
accident, or surgery. Males who have severe hemophilia may bleed heavily after circumcision.
Bleeding can occur on the body's surface (external bleeding) or inside the body (internal bleeding).
Signs of external bleeding may include:
 Bleeding in the mouth from a cut or bite or from cutting or losing a tooth
 Nosebleeds for no obvious reason
 Heavy bleeding from a minor cut
 Bleeding from a cut that resumes after stopping for a short time
Signs of internal bleeding may include:
 Blood in the urine (from bleeding in the kidneys or bladder)
 Blood in the stool (from bleeding in the intestines or stomach)
 Large bruises (from bleeding into the large muscles of the body)
Bleeding in the Joints
Bleeding in the knees, elbows, or other joints is another common form of internal bleeding in people who have hemophilia. This
bleeding can occur without obvious injury.
At first, the bleeding causes tightness in the joint with no real pain or any visible signs of bleeding. The joint then becomes
swollen, hot to touch, and painful to bend.
Swelling continues as bleeding continues. Eventually, movement in the joint is temporarily lost. Pain can be severe. Joint
bleeding that isn't treated quickly can damage the joint.
Bleeding in the Brain
Internal bleeding in the brain is a very serious complication of hemophilia. It can happen after a simple bump on the head or a
more serious injury. The signs and symptoms of bleeding in the brain include:
 Long-lasting, painful headaches or neck pain or  Sudden weakness or clumsiness of the arms or legs
stiffness or problems walking
 Repeated vomiting  Double vision
 Sleepiness or changes in behavior  Convulsions or seizures

DIAGNOSTIC EVALUATION

1. Coagulations Study

(A) Partial Thromboplastin Time (PTT)

 PTT is the blood test that looks how long it takes for blood clot. It can help to tell if you have bleeding
or clotting problem.
 If the patient in heparin medication, you will be watched for sign of bleeding.

(B)Thrombin Time

 TT is the blood test which measures the time it take for a clot to form in the plasma from a blood
sample in anticoagulant which had added an excess of thrombin.
 This test repeated with pooled plasma from normal patient. Different in time between the test and the
normal indicates an abnormality in the conversion of fibrogen.
 (C)Serum Platelet Level

 It is the test to measure how many platelet you have in your blood. Platelet also can help the blood
clot.
 The size is smaller than red and white blood cell.

2. Factor Assay

 Factor viii deficiency or extrinsic ( protein ) is an inherited disorder in which a lack of plasma protein.
 Factor viii leads to abnormal bleeding and it occurs when the body does not have enough of factor vii.
 It is very important blood clotting protein.

3. Amniocentesis

 The procedure that use to diagnose fetal defects in the early second trimester of pregnancy

 It is surrounds a fetus of womb

 Performed on fetal cells found in the sample can reveal the presence of many type of genetic disorders.

MANAGEMENT
Treatment with Replacement Therapy
The main treatment for hemophilia is called replacement therapy. Concentrates of clotting factor VIII (for hemophilia A)
or clotting factor IX (for hemophilia B) are slowly dripped or injected into a vein. These infusions help replace the clotting factor
that's missing or low.
Clotting factor concentrates can be made from human blood. The blood is treated to prevent the spread of diseases, such
as hepatitis.
Have replacement therapy on a regular basis to prevent bleeding. This is called preventive or prophylactic therapy.
Or, replacement therapy is done to stop bleeding when it occurs. This use of the treatment, on an as-needed basis, is
called demand therapy.
Complications of Replacement Therapy
Complications of replacement therapy include:

 Developing antibodies (proteins) that attack the clotting factor

 Developing viral infections from human clotting factors

 Damage to joints, muscles, or other parts of the body resulting from delays in treatment

 .
Home Treatment With Replacement Therapy
Both preventive (ongoing) and demand (as-needed) replacement therapy can be done at at home. Home treatment has several
advantages:

 Quicker treatment when bleeding happens. Early treatment lowers the risk of complications.

 Fewer visits to the doctor or emergency room are needed.

  Home treatment costs less than treatment in a medical care setting.

 Home treatment helps children accept treatment and take responsibility for their own health.
Other Types of Treatment
Desmopressin
Desmopressin (DDAVP) is a man-made hormone used to treat people who have mild hemophilia A. DDAVP isn't used
to treat hemophilia B or severe hemophilia A.
DDAVP stimulates the release of stored factor VIII and von Willebrand factor; it also increases the level of these
proteins in your blood. Von Willebrand factor carries and binds factor VIII, which can then stay in the bloodstream longer.
DDAVP usually is given by injection or as nasal spray.
Antifibrinolytic Medicines
Antifibrinolytic medicines (including tranexamic acid and epsilon aminocaproic acid) may be used with replacement
therapy. They're usually given as a pill, and they help keep blood clots from breaking down.
These medicines most often are used before dental work or to treat bleeding from the mouth or nose or mild intestinal
bleeding.
Gene Therapy
Researchers are trying to find ways to correct the faulty genes that cause hemophilia. Gene therapy hasn't yet developed
to the point that it's an accepted treatment for hemophilia.
Treatment of a Specific Bleeding Site
Pain medicines, steroids, and physical therapy may be used to reduce pain and swelling in an affected joint. Talk with
your doctor or pharmacist about which medicines are safe for you to take.
NURSING MANAGEMENT
Nursing Diagnosis
1. Ineffective body protection related to lack of clotting factor
Goal
 Increasing patient body protection
Interventions
 Asses patient body protection by taking CBC to evaluate patient condition
 Instruct patient on bleeding precaution to promote early intervention to prevent injury
 Assist with administration of factor concentration, fresh frozen plasma, cryoprecipitate or blood to treat acute of
bleeding.
 If bleeding, apply cold compress at bleeding site to help slow bleeding
 Avoid any route of injection ( IM, IV, Subcutaneous ) or rectal medication that cause bleeding into tissue
2. Risk of aspiration related to uncontrolled nose bleeding.
Goal
 Reduce risk of aspiration, Control nose bleeding
Interventions
 Asses patient nose bleeding to evaluate patient condition
 Apply cold compress to reduce nose bleeding
 Avoid patient from expose with high temperature to avoid nose bleeding
 Avoid patient in doing major surgery to avoid excessive bleeding (aspiration)
 Replace clotting factor and blood product to increase patient blood clotting.
 Avoid all anticoagulant medication ( Heparin, Aspirin )to control excessive bleeding.
3. Pain related bleeding into tissue
Goal
 Patient will verbalize that pain is relieved to a satisfactory level
Interventions
 Asses patient pain by report the location, intensity, and rate of pain (pain scale) to provide caregiver with data for
treatment plan.
 Administer opiod (morphine) as prescribe to control pain from severe to moderate.
 Avoid IM injection because the risk of bleeding into the muscle which can cause more pain
 Reassess the level of pain within 1 hour after administer opiod to determine the effectiveness of treatment ordered.
 Monitor sedation and respiratory status of the patient receiving opiod of pain because opiod can cause depress respiratory
center of the brain

Ongoing Care

 Follow your treatment plan exactly as your doctor prescribes.

  Have regular checkups and vaccinations as recommended.

 Tell all of your health care providers—such as your doctor, dentist, and pharmacist—that you have hemophilia. You also
may want to tell people like your employee health nurse, gym trainer, and sports coach about your condition.

 Have regular dental care. Dentists at the HTCs are experts in providing dental care for people who have hemophilia. If
you see another dentist, tell him or her that you have hemophilia.

 Know the signs and symptoms of bleeding in joints and other parts of the body. Know when to call your doctor or go to
the emergency room. For example, you'll need care if you have:

o Heavy bleeding that can't be stopped or a wound that continues to ooze blood.

o Any signs or symptoms of bleeding in the brain. Limited motion, pain, or swelling of any joint.
Nursing Diagnosis and Interventions for Hemophilia
1. Ineffective Tissue Perfusion  related to : active bleeding as evidenced by decreased consciousness, bleeding.
Expected outcomes: There was no impairment of consciousness, good capillary refill, bleeding can be resolved
Nursing Interventions
1. Assess the cause of bleeding
Rational : By knowing the cause of bleeding it will assist in determining appropriate interventions for patients
2. Assess skin color, hematoma, cyanosis
Rational : Provide information about the degree / adequacy of tissue perfusion and assist in determining appropriate intervention
3. Collaboration in the provision of adequate IVFD
Rational : Maintain fluid and electrolyte balance and maximize contractility / cardiac output so that the circulation becomes
inadequate
4. Collaboration in the provision of blood transfusion.
Rational: Repair / menormalakan red blood cell count and enhance oxygen-carrying capacity to be adequate tissue perfusion.
2. Fluid Volume Deficit  related to : loss due to bleeding as evidenced by a dry oral mucosa, skin turgor is slow again.
Expected outcomes: Indicates repairs fluid balance, moist oral mucosa, skin turgor quickly returned less than 2 seconds
Nursing Interventions:
1. Monitor vital signs
Rational : Changes in vital signs may indicate the direction of abnormal fluid loss due to an increase in bleeding / dehydration
2. Monitor output and income
Rational : Need to determine kidney function, fluid replacement needs and to help evaluate the fluid status
3. Estimate the wound drainage and the loss of a visible
Rational : Provide information about the degree of hypovolemia and help determine intervention
4. Collaboration in the provision of adequate fluid
Rational : Maintain fluid balance due to bleeding
3. Risk for Injury related to : weakness of the defense secondary to hemophilia as evidenced by frequent injuries
Expected outcomes: injury and complications can be avoided / did not happen.
Nursing Interventions
1. Maintain security of client's bed, put a safety on the bed
Rational : Fragile tissue and impaired clotting mechanisms boost the risk of bleeding despite the injury / mild trauma
2. Avoid injury, light - weight
Rational : Patients with hemophilia are at risk of spontaneous bleeding was controlled so that the required monitoring every
move that allows the occurrence of injury
3. Keep an eye on every move that allows the occurrence of injury
Rational : Early identification and treatment can limit the severity of complications
4. Encourage the parents to bring children to the hospital immediately in case of injury
Rational : Parents can find out mamfaat of injury prevention / risk of bleeding and avoid injury and complications.
5. Explain to parents the importance of avoiding injury.
Rational : Lower the risk of injury / trauma.
It's a good idea to keep a record of all previous treatments. Be sure to take this information with you to medical appointments and
to the hospital or emergency room.
Recruitment For Staff Nurse In Himachal Pradesh Subordinate Services Selection Board – Himachal Pradesh
NOVEMBER 22, 2013

Himachal Pradesh Subordinate Services Selection Board (HPSSSB)

Staff Nurse
Himachal Pradesh Subordinate Services Selection Board

Address: Secretary, Himachal Pradesh Subordinate Services Selection Board

Postal Code: 177001

City Hamirpur

State Himachal Pradesh

Pay Scale: Rs. 10300-34800+3200 GP

Educational Requirements: Should 10+2 class pass preferably with science from a recognized board of school
education/university.

Qualifications: Should be qualified “A” Grade nurse or should be BSC Nursing pass from a recognized university/institution.

Details will be available at: http://himachal.nic.in/hpsssb/PDF/Vacancy21Nov2013_A1b.pdf

No of Post: 140 Posts

How To Apply: Applications on the prescribed Proforma, in sealed covers, are invited for the following posts under the
Government of Himachal Pradesh so as to reach the Secretary, Himachal Pradesh Subordinate Services Selection Board,
Hamirpur (Himachal Pradesh) PIN-177001 on or before 21.12.2013 However, for the candidates residing in Lahaul & Spiti
District, Kinnaur District, Pangi & Bharmour Sub-Divisions of Chamba District and Dodra Kwar Sub-Division of Shimla District
of Himachal Pradesh, the last date for receipt of applications is 06.01.2014 The applications received after these dates will not be
entertained. The Board will not be responsible for delay in receipt of applications due to any reasons what so ever.

Last Date: 21.12.2013
For more Government Jobs visit Sarkari Naukri

Grossly, BPH consists of variably sized nodules that are soft or firm, rubbery, and yellow-gray, and bulge from the cut surface
upon transection (Fig. 6.2). If there is prominent epithelial hyperplasia in addition to stromal hyperplasia, the abundant luminal
spaces create soft and grossly spongy nodules that ooze a pale-white watery fluid. If BPH is predominantly fibromuscular, there
may be diffuse enlargement or numerous trabeculations without prominent nodularity Degenerative changes include calcification
and infarction. BPH usually involves the transition zone, but occasionally nodules arise from the periurethral tissue at the bladder
neck. Protrusion of bladder neck nodules into the bladder lumen is referred to as median lobe hyperplasia (Fig. 6.2). Rarely,
hyperplastic nodules are present in the peripheral zone. Microscopically, BPH is invariably nodular, comprising varying
proportions of epithelium, fibrous connective tissue, and smooth muscle. There are five types of nodules, including
adenomyofibromatous (most common), fibromuscular, muscular (uncommon), fibroadenomatous, and stromal (Fig. 6.3). In
practice, pathologists do not subclassify BPH histologically because of the wide variation in composition. Common associated
findings include chronic inflammation, acinar atrophy, and luminal corpora amylacea and microcalculi. The transition zone is
infrequently sampled by needle biopsy unless the urologist specifically targets this area or there is massive BPH, which
compresses the peripheral zone. The diagnosis of BPH is often used by pathologists in reporting the findings in needle biopsy
specimens when only normal benign peripheral zone tissue is present. However, such findings should be referred to as 'benign
prostate tissue' as histologic biopsy results do not correlate with BPH, except when stromal nodules are present.(25) We require
the presence of at least part of a nodule for the diagnosis of BPH. Narrow 18-gauge biopsies virtually never contain the entire
nodule unless it is very small and fortuitously sampled. Casual use of the term BPH for benign prostatic tissue may mislead the
urologist into believing that a palpable nodule or hypoechoic focus of concern has been sampled and histologically evaluated; it
is important for the pathologist to correlate the light microscopic findings with the clinical impression, so communication with
the urologist is vital.
Vascular insufficiency probably accounts for infarction of BPH nodules, seen in up to 20% of resected cases (Fig. 6.3). The
center of the nodule undergoes hemorrhagic necrosis, often with reactive changes in the residual epithelium at the periphery,
including squamous metaplasia and transitional cell metaplasia.

Vasan Eye Care Hospital

(0712) 3989000, 9595743736
46,Ramson Tower, Congress Nagar ,T Point, Humpyard Road, Dhantoli, Nagpur - 440012
Eye Hospitals , Private Hospitals

Soods Eye Hospital For Advance Eyec...

(0712) 2435535, 9326992999
Shree Wardhan Complex, Near Big Bazaar, Wardha Road, Panchsheel Square, Nagpur - 440012

Samarth Netralaya
(0712) 2722234, 9890374024
Shri Guru Darshan Complex, Near Chhapru Nagar Square, Central Avenue, Chhapru Nagar, Nagpur - 440008
Eye Hospitals , Eye Clinics

Mahatme Eye Bank & Eye Hospital

(0712) 2289101
2163-C, Chintaman Nagar, Somalwada, Nagpur - 440015
Eye Hospitals , Hospitals

Evista Eye Care Centre

(0712) 2467415
Opp Dhantoli Park, Dhantoli, Nagpur - 440012
Eye Hospitals , General Physician Doctors

DR.PANJABRAO DESHMUKH NURSING INSTITUTE, AMRAVATI

MID-TERM EXAMINATION

R.G.N.M 1ST YEAR-2013 TIME- 10AM -1PM

COMMUNITY HEALTH NURSING MARK-75

Q1.A) SELECT THE MOST APPROPRIATE ANSWER FROM THE FOLLOWING. (05)

a) The effective method of health education is--------

1) Group discussion 2) lecture 3) Exhibition 4) One way
b) For disinfection of well 1000 lit. of water required ----- gm bleaching powder.
1)2.5 2)3.5 3)4.5 4)2.8
c) The daily requirement of calcium is------- mg in adult
1)10 2)50 3)100 4)500
d)Chemical name for vit.c is-------
1) retinol 2) ascorbic acid 3)folic acid 4) none of these
e)Protective foods are rich in------------
1) carbohydrate 2) vit., minerals,protein 3) fat 4) none of these

C) STATE TRUE OR FALSE (05)

1) yellow fruits are rich in vit. B complex-----

2)1 gm of fat contain 4 calories---------------

3) perfect functioning of the body and mind is called as positive health-----

4)hard watert produces good foam of soap………….

5)chlorination is one of the important method of purification of water------

 DR.PANJABRAO DESHMUKH NURSING INSTITUTE, AMRAVATI

MID-TERM EXAMINATION

R.G.N.M 2nd YEAR-2013

TIME- 10AM -1PM TOTAL MARKS : 75

-------------------------------------------------------------------------------------------------------------------------------

Q1) A. select the most appropriate answer from the following. (5)

1) Delusion is disturbances of…………….

a) Perception b) Insight c) Ego d) Thought

2) An absolute contraindication for E.C.T is…………………

a) Raised I.C.P b) pulmonary diseases c) C.C.F d) recent stroke

3) ………………is not a characteristic of mental illness.

a) Abnormal behavior b) phobia c) sound sleep d) hallucination

4) Hallucination is disturbance of…………………..

a) Memory b) perception c) insight d) thinking

5) ………………..is the reality state of mind.

a) Ego b) super ego c) ID d) amnesia

B. match the followings. (5)

1) Indicator of mental health a)schizophrenia

2) Repetition of words b) sexual identity
3) Functional psychosis c) broad opening
4) Phallic stage d) perception of reality
5) Communication technique e) echolalia
f) echopraxia

C. True or false. (5)

1) mental ill person should only be treated as prisoner.
2) Faulty & fixed thoughts are delusional thought.
3) false perception of the object is called hallucination.
4) E.C.T is important in the treatment of neurosis.
5) Delusional of grandiosity is seen in schizophrenia.

Q2) Differentiate between any TWO of the followings. (10)

a) Psychosis & Neurosis

b) Mental illness & mental health
c) Component & indicator of mental health
d) Delusion & hallucination
Q3) write short notes on ( any four) (20)

a) Etiology of mental illness

b) Hallucination
c) Principles of psychiatric nursing
d) Defence mechanism
e) Characteristic of mentally healthy person

Q4) answer the followings. (any two) (30)

a) Define therapeutic nurse patient relationship. (02)

b) Write the goals of nurse patient relationship. (03)
c) Explain the phases of nurse patient relationship. (05)
d) Explain the techniques used in nurse patient relationship. (05)

Q5) a) Define the Electroconvulsive therapy (02)

b) Write the different types of E.C.T (02)
c) Write the side effects of E.C.T (03)
d) What are the indication & contraindication of E.C.T (03)
e) Write the role of nurse in pre & post E.C.T procedure. (05)

Q6) a) Define schizophrenia & its type (03)

b) What are the causes & symptoms of schizophrenia (04)
c) What are the therapies use in schizophrenia (03)
d) Write the nursing management. (05)