HEMATOLOGIC SYSTEM
The hematologic system consists of the blood and the sites where
blood is produced, including the bone marrow and the
reticuloendothelial system (RES).
Reticuloendothelial System (RES)
- Composed of special tissue macrophages
- The remove old or damaged cells from the circulation
- They stimulate the inflammatory process and present
antigens to the immune system
Blood
- Blood is a specialized organ that differs from other organs in that
it exists in a fluid state.
- Blood is composed of plasma and various types of cells.
- Plasma is the fluid portion of blood; it contains various proteins,
such as albumin, globulin, fibrinogen, and other factors
necessary for clotting, as well as electrolytes, waste products,
and nutrients.
- About 55% of blood volume is composed of plasma
The Blood and Its Components
It is composed of plasma, white blood cells,
platelets, and red blood cells
- Plasma is the most mature form of B
lymphocyte
- WBC: fights infection
- Platelets
o Fragment of megakaryocyte
o Provides basis for coagulation to occur
o Maintain hemostasis
o Average lifespan is 10 days
- RBC (erythrocyte)
o Carries hemoglobin to provide oxygen to tissues
o Average lifespan is 120 days
Let’s Test Your Stock Knowledge on Hematologic Systems
1. What do you call the fluid portion of the blood which is mostly
made up of water? Plasma
2. What do you all the portion of the blood where antibodies are
suspended? Plasma/Serum
3. What gives red blood cells their color? It is the pigment of RBCs.
Hemoglobin
4. What do you call the percentage of the blood occupied by red
blood cells? Hematocrit
5. What plasma protein produced by the liver is responsible for
oncotic pressure or colloid osmotic pressure? Albumin
HEMATOPOIESIS
- Complex process of formation and maturation of blood cells
- Primary site for hematopoiesis is the bone marrow
Test your Knowledge. True or False
1. What plasma proteins synthesized by the liver is
responsible for clot formation? Fibrinogen and
prothrombin
2. Clients with hepatic failure have dependent edema and
signs and symptoms of bleeding. True
3. Where is the site of blood cell formation? Red Bone
Marrow
4. Where is the red bone marrow found. Flat bones
BONE MARROW
- Site of hematopoiesis or blood cell formation
- One of the largest organs of the body, making up 4% to 5%
of total body weight
- In adults, blood cell formation is usually limited to the
pelvis, ribs, vertebrae, and sternum
Let’s test your knowledge
1. Bone Marrow depression leads to decrease in RBCs, WBCs,
and PLTs. True
2. What do you call the medical term for an overall decrease
in RBCs, WBCs, and PLTs? Pancytopenia
3. What is the most preferred site for bone marrow
aspiration? Posterior Iliac Crest
ERYTHROCYTES/RBCs
- Mature erythrocytes consist primarily
of hemoglobin, which contains iron
and makes up 95% of the cell mass.
- The presence of a large amount of
hemoglobin enables the red cell to
perform its principal function, which is
the transport of oxygen between the lungs and tissues.
Let’s test your knowledge
1. What is the medical term for an abnormally low RBC count?
Anemia
2. Clients with anemia have fatigue as manifestation. True
3. What do you call the substance found in red blood cells that
carries oxygen and carbon dioxide to and from the tissues?
Hemoglobin
4. Clients who have low hemoglobin may have a ruddy
complexion False
5. Clients who have low hemoglobin may have dyspnea True
6. What important mineral is needed in the synthesis of
hemoglobin? Iron
Vitamin B12 and Folate Metabolism
- Required for the synthesis of deoxyribonucleic acid (DNA)
in RBCs
- Both vitamin B12 and folate are derived from the diet.
- Folate is absorbed in the proximal small intestine
- The vitamin B-12 intrinsic factor complex is absorbed in the
distal ileum
- But if there is only small amount stored in the body and if
the diet is also deficient in folate, then the stores in the
body will quickly become depleted
Red Blood Cell Destruction
- Average lifespan of a normal circulating erythrocyte is 120
days
- Aged erythrocytes lose their elasticity and become trapped
in small blood vessels in the spleen
 - Removed from the blood by the reticuloendothelial cells,
particularly in the liver and the spleen
- Erythrocytes are destroyed, most of their hemoglobin is
recycled
- Hemoglobin also breaks down to form bilirubin which is
then secreted in the bile
- Most of the iron is recycled to form new hemoglobin
molecules within the bone marrow
- Small amounts are lost daily in the feces and urine and
monthly in menstrual flow
Let’s test your stock knowledge
1. What do you call the hormone responsible for the
production of RBCs. Erythropoietin
2. Clients with renal failure have decreased with RBC count.
True
3. What do you call the medication given to clients with renal
failure to increase their RBC counts? Epoietin Alfa (epogen)
4. What do you call the medical term for an elevated RBC
count? Polycythemia
5. Clients with polycythemia have a ruddy skin complexion
True
6. How many days is the average life span of RBCs? 120 days
7. What organ is known as the graveyard of RBCs? Spleen
ASSESSMENT
Health History
- A careful health history and physical assessment can
provide important information related to a patient’s known
or potential hematologic diagnosis
1. Ethnicity
o Because many hematologic disorders are more
prevalent in certain ethnic groups, assessments of
ethnicity and family history are useful
2. Family history
3. Nutritional history
o Similarly, obtaining a nutritional history and
assessing the use of prescription and over-the-
counter medications, as well as herbal
supplements, are important to note, because
several conditions can result from nutritional
deficiencies, or from the use of certain herbs or
medications.
Physical Assessment
The physical assessment should be comprehensive and include
careful attention to the following organs. These are very important
especially in relation to the hematological disorders:
- Skin
- Oral Cavity
- Lymph nodes
- Spleen
LABORATORY TEST
Diagnostic Evaluation
1. CBC
- identifies the total number of blood cells (leukocytes,
erythrocytes, and platelets) as well as the hemoglobin,
hematocrit (percentage of blood volume consisting of
erythrocytes), and RBC indices.
2. Peripheral Blood Smear
- this test, a drop of blood is spread on a glass slide, stained,
and examined under a microscope.
- The shape and size of the erythrocytes and platelets, as well
as the actual appearance of the leukocytes, provide useful
information in identifying hematologic conditions.
- Blood for the CBC is typically obtained by venipuncture
3. Coagulation Studies (PT, aPTT, PTT, INR, and Clotting time)
- INR and aPTT
o The INR and aPTT serve as useful screening tools
for evaluating a patient’s clotting ability and
monitoring the therapeutic effectiveness of
anticoagulant medications.
o In both tests, specific reagents are mixed into the
plasma sample, and the time taken to form a clot
is measured.
o For these tests to be accurate, the test tube must
be filled with the correct amount of the patient’s
blood; either excess or inadequate blood volume
within the tube can render the results inaccurate.
4. Serum fibrinogen
- Evaluates the part of the clotting process in which soluble
fibrinogen is converted into fibrin threads
5. D-Dimer Assay
- Used to rule out the presence of inappropriate blood clot or
thrombus
6. Coomb’s test
- Used to detect antibodies that are stuck to the surface of
red blood cells
7. Fibrin degradation products
- Specific test that measures the amount of FDP in the blood
and also known as fibrin plate products or FSP test or fibrin
breakdown products test
8. Serum Ferritin
- Helps to check how much iron in your body stores
9. Total Iron Binding Capacity
- Blood test to see if you have too much or too little iron in
your blood
BONE MARROW ASPIRATION AND BIOPSY
- Used to document infection or tumor within the marrow
- Other specialized tests can be performed on the marrow
aspirate, such a cytogenetic analysis or
immunophenotyping
o Immunophenotyping: identifying specific
proteins expressed by cells which are useful in
further identifying certain malignant conditions
and, in some instances, establishing a prognosis.
Preparations:
- Careful explanation of the procedure
o May be done at the patient’s bedside or in the
outpatient setting
- Antianxiety agent may be prescribed
o Patients may be anxious
- Describe and explain to the patient the procedure and the
sensations that will be experienced
- Risks, benefits, and alternatives are also discussed
- Signed informed consent is needed
Before Aspiration:
- Skin is cleansed using aseptic technique
- Small area is anesthetized with a local anesthetic agent
through the skin and subcutaneous tissue to the
periosteum of the bone. It is not possible to anesthetize the
bone itself.
- The bone marrow needle is introduced with a stylet in
place.
NONMALIGNANT HEMATOLOGIC DISORDERS - the released hemoglobin is converted in large part to bilirubin
1. ANEMIA and, therefore, the bilirubin concentration rises.
- Hemoglobin concentration is lower than normal - The increased erythrocyte destruction leads to tissue hypoxia,
- it reflects the presence of fewer than the normal number of which in turn stimulates erythropoietin production.
erythrocytes (i.e., red blood cells [RBCs]) within the circulation. - This increased production is reflected in an increased
- As a result, the amount of oxygen delivered to body tissues is reticulocyte count as the bone marrow responds to the loss of
also diminished. erythrocytes.
- Anemia is not a specific disease state but a sign of an underlying - Increased erythrocyte destruction leads to tissue hypoxia
disorder.
- Most common hematologic condition SICKLE-CELL DISEASE (SCD)
- SCD can cause a severe hemolytic anemia that results from
CAUSES AND TYPES OF ANEMIA inheritance of the sickle hemoglobin (HbS) gene, which
a. Hypoproliferative anemias causes the hemoglobin molecule to be defective
- Bone marrow does not produce adequate numbers of - Patients with SCD are unusually susceptible to infection
erythrocytes particularly pneumonia and osteomyelitis
- May results from marrow damage due to medications - Complications include: infection, stroke, kidney injury,
- Decreased erythrocyte production is reflected by a low or impotence, and pulmonary hypertension
inappropriately normal reticulocyte count. Manifestations:
- Inadequate production of erythrocytes may result from marrow 1. Sickle-Cell Crisis
damage due to medications (e.g., chloramphenicol a. Acute vaso-occlusive crisis
[Chloromycetin]), chemicals (e.g., benzene), or from a lack of i. Most common and very painful
factors (e.g., iron, vitamin B12, folic acid, erythropoietin) ii. Results from entrapment of
necessary for erythrocyte formation. erythrocytes and leukocytes from
i. Iron Deficiency anemia the microcirculation causing tissue
o Results when the intake of dietary iron is inadequate for hypoxia, inflammation, and
hemoglobin synthesis necrosis due to inadequate blood
o The most common cause of iron deficiency anemia in men flow to specific region of tissue or
and postmenopausal women is bleeding from ulcers, organ
gastritis, inflammatory bowel disease, or GI tumors. b. Aplastic crisis
o The most common causes of iron deficiency anemia in i. Results from infection with human
premenopausal women are menorrhagia (i.e., excessive parvovirus
menstrual bleeding) and pregnancy with inadequate iron ii. hemoglobin levels fall rapidly and
supplementation marrow cannot compensate as
ii. Anemias in Renal Disease evidenced by absence of
o Anemia in patients with chronic kidney disease (CKD) reticulocytes
iii. Anemia of Inflammation c. Sequestration crisis
o Previously referred to as anemia of chronic i. Results when other organs pool
disease the sickle cells
o These disorders include rheumatoid arthritis; severe, ii. Common organs involved in adults
chronic infections; and many cancers. are liver and more seriously, the
o It is therefore imperative that the “chronic disease” be lungs
diagnosed when this form of anemia is identified so that it 2. Acute Chest Syndrome
can be appropriately managed - Manifested by fever, respiratory distress like tachypnea,
iv. Aplastic Anemia cough, wheezing, and new infiltrates are seen in chest xray
o Aplastic anemia is a rare disease caused by a decrease in or - Main cause of death in young adults with SCD
damage to marrow stem cells, damage to the
microenvironment within the marrow, and replacement of 3. Pulmonary Hypertension
the marrow with fat. - Manifestation of SCD
o Occurs when a medication or chemical is ingested - Common cause of death
in toxic amounts - Diagnosing is difficult since clinically symptoms rarely occur
v. Megaloblastic Anemia until damage is irreversible
o Caused by deficiencies of Vitamin B12 or folic acid - Patients may complain of fatigue, dyspnea on exertion,
o In the anemias caused by deficiencies of vitamin B12 or folic chest pain or syncope
acid, identical bone marrow and peripheral blood changes - Pulse ox measurements are normal, breath sounds are clear
occur because both vitamins are essential for normal DNA to auscultation until disease progresses to later stages
synthesis.
o Megaloblastic anemias develop slowly like months so body 4. Stroke
cannot compensate well for a long time - ischemic: most common and particularly in young children
o Symptoms of anemia may not develop until the anemia is and older adults
very severe - Hemorrhagic stroke: most common in younger adults

b. Hemolytic anemias 5. Reproductive Problems

- Premature destruction of erythrocytes results in the liberation of - Almost 25% of men with SCD develop hypogonadism
hemoglobin from the erythrocytes into the plasma; - Low testosterone problems, low libido, erectile
dysfunction, and infertility can also occur

THALASSEMIAS
- Group of hereditary anemia characterized by hypochromia
(abnormal decrease in hemoglobin content of erythrocytes)
and also characterized by microcytosis (smaller than normal
erythrocytes)
- Characterized by hemolysis and varying degrees of anemia
a) Alpha Thalassemias
- Occurs mainly in people from Asia and middle east
b) Beta Thalassemias
- Most prevalent in Mediterranean regions and can occur in
people from Asia and middle east
GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY
- The G-6-PD gene is the source of the abnormality in this
disorder
o Gene produces an enzyme inside erythrocyte
which is essential for membrane stability
- Diagnosed by a screening test for the deficiency or by a
quantitative assay of G-6-PD
IMMUNE HEMOLYTIC ANEMIAS
- Can result from exposure of the erythrocyte to antibodies
- Alloantibodies result from immunization of person with
foreign antigens
- Example of alloimmune hemolytic anemia in adults is
anemia that results from a hemolytic transfusion reaction
HEREDITARY HEMOCHROMATOSIS
- A genetic condition in which excess iron is absorbed from
the GI tract
- Actual prevalence of hemochromatosis is lower among
Asian Americans, African Americans, Latinos, and Pacific
islanders but higher in European descent
- Women are less often affected than men because women
lose iron through menses
c. Bleeding
- Refer to the table for more of the parts on the types of
anemia
CLINICAL MANIFESTATIONS OF ANEMIA
- Profound vascular collapse
- In general, the more rapidly an anemia develops, the more
severe its symptoms (Bunn, 2017). An otherwise healthy
person can often tolerate as much as a 50% gradual
reduction in hemoglobin (e.g., over months) without
pronounced symptoms or significant incapacity, whereas
the rapid loss of as little as 30% (e.g., over minutes) may
precipitate profound vascular collapse in the same person.
- Slight tachycardia on exertion and possibly fatigue
- person who has become gradually anemic, with
hemoglobin levels between 9 and 11 g/dL, usually has fewer
or no symptoms other than slight tachycardia on exertion
and possibly fatigue
- Very active people are more likely to have symptoms, and those
symptoms are more likely to be pronounced than in more
sedentary people
- Hypothyroidism with decreased oxygen needs may be
completely asymptomatic, without tachycardia or increased
cardiac output, at a hemoglobin level of 10g/dL
- Coexistent cardiac, vascular, or pulmonary disease may develop
more pronounced symptoms of anemia (dyspnea, chest pain,
muscle pain, or cramping)
ASSESSMENT AND DIAGNOSTIC FINDINGS
- CBC
- Iron studies (serum iron level, total iron-binding capacity [TIBC],
percent saturation, and ferritin
- Bone marrow aspiration may be performed
COMPLICATIONS
Patients with underlying heart disease are far more likely to have
angina or symptoms of heart failure than those without heart disease.
- Heart failure
- Angina
- Paresthesias
- Confusion
- Injury related to falls
- Depressed mood
- Delirium
NURSING INTERVENTIONS
- Managing fatigue: focusing on assisting the patient to prioritize
activities and establishing a balance between activity an rest that
is acceptable to the patient
- Maintaining adequate nutrition: A healthy diet should be
encouraged. Nurse should inform patient that alcohol interferes
with utilization of essential nutrients and advise moderation in
the intake of alcoholic beverages There should be dietary
consultations involving family members and cultural aspects
related to food preferences and food preparation. Dietary
supplements like vitamins iron folate and protein may be
prescribed
- Activity intolerance: monitors the patient’s vital signs. Monitor
Pulse ox reading closely. Other meds such as antihypertensive
meds may be adjusted and withheld
- Promoting adherence with prescribed therapy: assists the
patient to develop ways to incorporate the therapeutic plan into
everyday activities rather than merely giving patient a list of
instructions
- Monitoring and managing potential complications: In
megaloblastic forms of anemia, then the complication is
neurologic and so, neurologic assessment must be performed for
patients with known or suspected megaloblastic anemia
2. POLYCYTHEMIA
- Refers to an increased volume of RBCs
- Hematocrit is elevated (more than 55% in males, more than
50% in females)
- Dehydration (decreased volume of plasma) can cause an
elevated hematocrit but not typically to the level to be
considered polycythemia.
 - Polycythemia is classified as either primary or secondary for the disorder are idiopathic thrombocytopenic purpura
polycythemia. and immune thrombocytopenia
o Primary polycythemia, also called polycythemia d. Platelet defects
vera, is a proliferative disorder o Quantitative
o Secondary polycythemia is caused by excessive ▪ thrombocytopenia, thrombocytosis
production of erythropoietin. This may occur in ▪ relatively common;
response to a reduced amount of oxygen, which o Qualitative
acts as a hypoxic stimulus, as in heavy cigarette ▪ however, qualitative defects can also
smoking, obstructive sleep apnea (OSA), chronic occur
obstructive pulmonary disease (COPD), or ▪ With qualitative defects, the number of
cyanotic heart disease, or with conditions such as platelets may be normal, but the
living at a high altitude or exposure to low levels platelets do not function normally
of carbon monoxide e. Hemophilia
o Secondary polycythemia can also occur from - Indistinguishable but distinguishable through lab tests
neoplasms (e.g., renal cell carcinoma) that o Hemophilia A
stimulate erythropoietin production, excess ▪ caused by a genetic defect that results
erythropoietin-stimulating agent use, or in deficient or defective factor VIII.
androgen use. o Hemophilia B
▪ Aka Christmas Disease
3. NEUTROPENIA ▪ stems from a genetic defect that causes
- A neutrophil count of less than 2000/mm3 deficient or defective factor IX.
- Results from decreased production of neutrophils or f. Von Williebrand Disease (vWD)
increased destruction of these cells - Usually inherited as a dominant trait, vWD is a common
- At increased risk for infection bleeding disorder that affects males and females equally.
o A patient with neutropenia is at increased risk for - The disease is caused by a deficiency of vWF or Von
infection from both exogenous and endogenous Williebrand Factor, which is necessary for factor VIII
sources activity. vWF is also necessary for platelet adhesion at the
site of vascular injury.
4. LYMPHOPENIA o Type 1
- Lymphocyte count is less than 5000 ▪ Most common
- Lymphopenia (a lymphocyte count less than 1500/mm3) ▪ Characterized by decreases in
can result from ionizing radiation, long-term use of structurally normal vWF
corticosteroids, uremia, infections (particularly viral o Type 2
infections), some neoplasms (e.g., breast and lung cancers, ▪ Shows variable qualitative defects
advanced Hodgkin disease), and some protein-losing based on the specific vWF subtype
enteropathies (in which the lymphocytes within the involved
intestines are lost) o Type 3
- Result from ionizing radiation, long-term use of ▪ Very rare (less than 5% of cases) and is
corticosteroids, uremia, infections, some neoplasms, and characterized by a severe vWF
some protein-losing enteropathies deficiency as well as significant
deficiency of factor VIII
5. BLEEDING DISORDERS
- Failure of normal hemostatic mechanisms can result in 6 ACQUIRED COAGULATION DISORDERS
bleeding, which may be severe. 1. Liver Disease
- Bleeding is commonly provoked by trauma; however, in - result in diminished amounts of the factors needed to
certain circumstances, it can occur spontaneously. When maintain coagulation and hemostasis
the cause is platelet or coagulation factor abnormalities, 2. Vitamin K Deficiency
the site of bleeding can be anywhere in the body. - The synthesis of many coagulation factors depends on
- When the source is vascular abnormalities, the site of
vitamin K.
bleeding may be more localized. Some patients have
- Vitamin K deficiency is common in malnourished patients
simultaneous defects in more than one hemostatic
mechanism - Prolonged use of some antibiotics decreases the intestinal
a. Secondary Thrombocytosis flora that produces vitamin K, depleting vitamin K stores.
- Increased platelet production is the primary mechanism of 3. Disseminated Intravascular Coagulation
secondary, or reactive, thrombocytosis. - Disseminated intravascular coagulation (DIC) is not an
b. Thrombocytopenia actual disease but a sign of an underlying condition. DIC
- Thrombocytopenia (low platelet level) can result from may be triggered by sepsis, trauma, cancer, shock, abruptio
various factors: decreased production of platelets within placentae, toxins, allergic reactions, and other conditions;
the bone marrow, increased destruction of platelets, or the vast majority (two thirds) of cases of DIC are initiated by
increased consumption of platelets (e.g., the use of an infection or a malignancy
platelets in clot formation). - The severity of DIC is variable, but it is potentially life-
c. Immune Thrombocytopenic Purpura (ITP)
threatening.
- ITP is a disease that affects people of all ages, but it is more
4. Thrombotic Disorders
common among children and young women. Other names
 - Thrombosis may occur as an initial manifestation of an
occult malignancy or as a complication from a pre-existing
cancer.
- It can also be caused by more than one predisposing factor.
5. Hyperhomocysteinemia
- Hyperhomocysteinemia can be hereditary, or it can result
from a nutritional deficiency of folate and, to a lesser
extent, of vitamins B12 and B6, because these vitamins are
cofactors in homocysteine metabolism
6. Antithrombin Deficiency
- most commonly a hereditary condition that can cause
venous thrombosis, particularly when the AT level is less
than 60% of normal.
- most common sites for thrombosis: deep veins of the leg
and the mesentery
7. Protein C Deficiency
- a vitamin K–dependent enzyme synthesized in the liver;
when activated, it inhibits coagulation.
- When levels of protein C are deficient, the risk of
thrombosis increases, and thrombosis can often occur
spontaneously.
- People who are deficient in protein C are often without
symptoms until their 20s; the risk of having a thrombotic
event then increases.
8. Protein S Deficiency
- When the level of protein S is deficient, this inactivation
process is diminished, and the risk of thrombosis can be
increased.
- Like patients with protein C deficiency, those with protein S
deficiency have a greater risk of recurrent venous
thrombosis early in life, and also with recurrent PE
9. Activated Protein C Resistance and Factor V Leiden Mutation
- APC resistance is a common condition that can occur with
other hypercoagulable states.
- APC is an anticoagulant, and resistance to APC increases the
risk of venous thrombosis.
- A molecular defect in the factor V gene has been identified
in most (90%) patients with APC resistance.
MALIGNANT HEMATOLOGIC DISORDERS
1. Clonal Stem Cell Disorders
- referred to as indolent neoplasms (where the increased
numbers of cells produced from a culprit clone all have the
same genotype)
2. Leukemia
- leukocytosis: refers to an increased level of leukocytes
(WBC) in the circulation
- common feature of the leukemias is an unregulated
proliferation of leukocytes in the bone marrow
- Classified according to the stem cell line involved, either
lymphoid (referring to stem cells that produce
lymphocytes) or myeloid (referring to stem cells that
produce nonlymphoid blood cells)
o also further classified as either acute or chronic,
based on the time it takes for symptoms to evolve
and the phase of cell development that is halted
- In acute leukemia, the onset of symptoms is abrupt, often
occurring within a few weeks. Leukocyte development is
halted at the blast phase, and thus most leukocytes are
undifferentiated cells or blasts. Acute leukemia can
progress rapidly, with death occurring within weeks to
months without aggressive treatment.
- In chronic leukemia, symptoms evolve over a period of
months to years, and the majority of leukocytes produced
are mature. Chronic leukemia progresses more slowly; the
disease trajectory can extend for years.
4 Types of Leukemia
a. Acute Myeloid Leukemia (AML)
- results from a defect in the hematopoietic stem cell that
differentiates into all myeloid cells: monocytes,
granulocytes (e.g., neutrophils, basophils, eosinophils),
erythrocytes, and platelets.)
- Most common non-lymphocytic leukemia
- AML develops without warning, with symptoms typically
occurring over a period of weeks.
Signs and Symptoms:
- Fever and infection result from neutropenia (low
- neutrophil count)
- weakness and fatigue, dyspnea on exertion, and pallor from
anemia
- petechiae, ecchymoses, and bleeding tendencies from
thrombocytopenia.
- Petechiae (pinpoint red or purple hemorrhagic spots on the
skin) or ecchymoses (bruises) are common on the skin
Diagnostic Findings:
- CBC: shows a decrease in both erythrocytes and platelets.
b. Chronic Myeloid Leukemia (CML)
- from a mutation in the myeloid stem cell
- average age at diagnosis is 64; it rarely occurs in children
- overall median life expectancy nearly equal to that of the
general population
- Patients may be asymptomatic, and leukocytosis is
detected by a CBC performed for some other reason.
- Three stages in CML: chronic, transformation, and
accelerated or blast crisis
- During the chronic phase, patients have few symptoms and
complications from the disease itself and problems with
infections and bleeding are rare.
- However, if the disease transforms to the acute phase (blast
crisis), the disease becomes more difficult to treat. Patients
develop more symptoms and complications as the disease
progresses.
c. Acute Lymphocytic Leukemia
- uncontrolled proliferation of immature cells (lymphoblasts)
derived from the lymphoid stem cell
- most common in young children with boys affected more
- often than girls; peak incidence is 4 years of age. After 15
years of age, it is relatively uncommon, until age 50 when
the incidence again rises
Clinical Manifestations:
- Manifestations of leukemic cell infiltration into other
organs are more common with ALL than with other forms
of leukemia
- pain from an enlarged liver or spleen and bone pain
d. Chronic Lymphocytic Leukemia
- common malignancy of older adults, most prevalent type of
adult leukemia in the Western world
- Unlike other forms of leukemia, a strong familial
predisposition exists with CLL; the disease can occur in 10%
 of those with a first- or second-degree relative with the
same diagnosis.
- derived from a malignant clone of B lymphocytes
- many patients are asymptomatic and are diagnosed
incidentally during routine physical examinations or during
treatment for another disease.
Nursing Interventions: LEUKEMIA
1. Nutritional intake is often reduced because of pain and
discomfort associated with stomatitis.
- Encouraging or providing mouth care before and after
meals and administering analgesic agents before eating can
help increase intake.
2. Small, frequent feedings of foods
3. Nutritional supplements are frequently used.
4. Parenteral nutrition may be required
5. Sponging with cool water may be useful, but cold water or ice
packs should be avoided
- because the heat cannot dissipate in constricted blood
vessels – useful for patients with fever
6. Assisting the patient to establish a balance between activity and
rest
7. Intake and output need to be measured accurately
8. Assessed for signs of dehydration as well as fluid overload
- with particular attention to pulmonary status and
development of dependent edema
9. Providing emotional support and discussing the uncertain future
3. Myelodysplastic Syndrome (MDS)
- group of clonal disorders of the myeloid stem cell that cause
dysplasia (abnormal development) in one or more types of
cell lines.
- most common feature of MDS – dysplasia of the
erythrocytes – is manifested as a macrocytic anemia and is
present in 80% to 90% of those with this disease
Signs and Symptoms:
- Some patients are asymptomatic, with the illness being
discovered incidentally when a CBC is performed for other
purposes.
- CBC: reveals a macrocytic anemia; leukocyte and platelet
counts may be diminished as well.
- fatigue is often present
Diagnosis:
- Official diagnosis of MDS is based on the results of a bone
marrow aspiration (to assess dysplasia) and biopsy (to
assess characteristics of the affected cells).
Medical Management:
- Patients frequently need repeated transfusions (red blood
cells [RBCs], platelets, or both) throughout the illness
trajectory to maintain adequate hemoglobin and platelet
levels (termed transfusion dependence).
Nursing Interventions: MDS
1. Education about infection risk (measures to avoid it, signs and
symptoms of developing infection, and appropriate actions to
take should such symptoms occur)
2. Patients receiving growth factor medications need instruction on
administering schedules and side effects
3. Chelation therapy – instructing the patient to take the
medication in the evening prior to dinner and gradually
increasing the dosage over time may diminish these side effects
- Chelation therapy is a process that is used to remove excess
iron acquired from chronic transfusions
4. Myeloproliferative Neoplasms
a. Polycythemia Vera
- sometimes called P vera or primary polycythemia, is a
proliferative disorder of the myeloid stem cells
- may evolve into myeloid metaplasia with myelofibrosis, or
AML
Clinical Manifestations:
- ruddy complexion and splenomegaly
- Symptoms: headache, dizziness, tinnitus, fatigue,
paresthesias, and blurred vision.
- Symptoms also result from increased blood viscosity may
also include angina, claudication, dyspnea, and
thrombophlebitis, particularly if the patient has
atherosclerotic blood vessels
Diagnostic Findings:
- elevated hemoglobin or hematocrit and either the presence
of a mutation of the JAK2 gene (which promotes cell
proliferation and a hypersensitivity to erythropoietin) or a
low serum erythropoietin level
Nursing Interventions: P Vera
1. Assess for symptoms carefully and regularly throughout the
illness trajectory
2. Nurse focuses on symptom management
- Fatigue is the most common symptom
3. Recommend bathing in tepid or cool water and avoiding
vigorous toweling off after bathing
4. Cocoa butter or oatmeal-based lotions and bath products or
baking soda dissolved in bathwater may also be helpful
b. Essential Thrombocythemia
- aka primary thrombocythemia, is a stem cell disorder
within the bone marrow.
- exact cause is unknown
- affects women twice as often as men and tends to occur
later in life (median age at diagnosis is 65 to 70 years)
Clinical Manifestations:
- many patients with ET are asymptomatic
- the illness is diagnosed as the result of finding an elevated
platelet count on a CBC. Symptoms occur most often when
the platelet count exceeds 1 million/mm3;
- When symptoms do occur, they primarily result from
vascular occlusion
- headaches are the most common neurologic
manifestations;
- other manifestations include transient ischemic attacks and
diplopia
- Tinnitus, paresthesias, or atypical chest pain may also occur
Complications:
- complications include inappropriate formation of thrombi
and hemorrhage
- A careful assessment of risk factors, history of peripheral
vascular disease, tobacco use, atherosclerosis, diabetes,
and prior thrombotic or bleeding events are all considered
in developing the treatment plan.
Nursing Interventions: ET
1. It is important to assess patients for a history of prior thrombotic
2. Needs to be educated about the risks of hemorrhage and
thrombosis
3. The patient is informed about s/s of thrombosis
- particularly the neurologic manifestations, such as visual
changes, numbness, tingling, and weakness
4. Patients taking aspirin should be informed about the importance
of taking this medication as well as the increased risk of bleeding
5. Patients taking hydroxyurea should have CBCs monitored
regularly
- the dosage is adjusted based on the platelet and WBC
count.
c. Primary Myelofibrosis
- aka agnogenic myeloid metaplasia or myelofibrosis with
myeloid metaplasia, is a chronic myeloproliferative
disorder that arises from neoplastic transformation of an
early hematopoietic stem cell.
- characterized by marrow fibrosis or scarring,
extramedullary hematopoiesis (typically involving the
spleen
- and the liver), leukocytosis, thrombocytosis, and anemia
- Cachexia, severe wasting, and massively enlarged liver and
spleen (hepatosplenomegaly) are seen in advanced
myeloproliferative disorders, particularly myelofibrosis.
Nursing Interventions: Primary Myelofibrosis
1. Small, frequent meals of foods that are high in calories and
protein
2. Energy conservation methods and active listening
3. Needs to be educated about s/s of infection
- bleeding, and thrombosis, as well as appropriate
interventions if these occur.
5. Lymphoma
- are neoplasms of cells of lymphoid origin
- usually start in lymph nodes but can involve in other
lymphoid tissues in the spleen, GI tract (e.g., the wall of the
stomach), liver, or bone marrow
- classified according to the degree of cell differentiation and
the origin of the predominant malignant cell
- classified into two categories: Hodgkin lymphoma and non-
Hodgkin lymphoma (NHL)
a. Hodgkin Lymphoma
- rare malignancy that has high cure rate
- It is somewhat more common in men than in women and
has two peaks of incidence: one from age 15 to 34 and the
other after 60 years of age
- the malignant cell of Hodgkin
lymphoma is the Reed-Sternberg
cell – a gigantic tumor cell that is
morphologically unique and
thought to be of immature
lymphoid origin; it is the
pathologic hallmark and essential diagnostic criterion.
Clinical Manifestations:
- usually begins an enlargement of one or more lymph nodes
on one side of the neck - The individual nodes are painless
and firm but not hard.
- most common sites for lymphadenopathy are the cervical,
supraclavicular, and mediastinal nodes; involvement of the
iliac or inguinal nodes or spleen is much less common.
Diagnostic Findings:
- diagnosis is made by means of an excisional lymph node
biopsy
Complications:
- Herpes zoster is a common complication in patients with
lymphoproliferative disease, such as Hodgkin lymphoma
- treatment involves a short
course (2-4 months) of
chemotherapy followed by
radiation therapy to the specific
involved area
Nursing Interventions: Hodgkin Lymphoma
1. Encourage patients to reduce factors that increase the risk of
developing second cancers
- such as use of tobacco and alcohol and exposure to
environmental carcinogens and excessive sunlight
2. Provide education about relevant self-care strategies and
disease management
b. Non-Hodgkin Lymphoma (NHL)
- heterogenous group of cancers that originate from the
neoplastic growth of lymphoid tissue
- sixth most common type of cancer in US; incidence rates
have almost doubled in the past 35 years
Clinical Manifestations:
- Lymphadenopathy is the most common
Diagnostic Findings:
- based on data obtained from CT and PET scans, bone
marrow biopsies, and occasionally cerebrospinal fluid
analysis
Nursing Interventions: NHL
1. Important for the nurse to know the specific disease type, stage
of disease, treatment history, and current treatment plan
2. Patients need to be educated to minimize the risks of infection,
to recognize signs of possible infection, and to contact their
primary provider if such signs develop
3. The nurse must know the tumor location so that assessments
can be targeted appropriately
6. Multiple Myeloma
- malignant disease of the most mature form of B lymphocyte
– the plasma cell.
- second most common hematologic cancer
- Staging is based on four criteria:
o serum albumin (presumed to be a negative acute
phase reactant),
o serum lactate dehydrogenase (an enzyme that, when
elevated, is associated with a poorer prognosis),
o serum beta-2 microglobulin (presumed to be an
indirect measure of tumor burden), and
o cytogenetic findings (where certain abnormalities are
associated with a poor prognosis)
Clinical Manifestations:
- classic presenting symptom of multiple myeloma is bone
pain - usually in the back or ribs
- Bone pain associated with myeloma increases with
movement and decreases with rest; patients may report
that they have less pain on awakening but more during the
day

**clinical manifestations of multiple myeloma and the mechanisms by
which they develop
** Those manifestations that
are reflective of “end-organ damage” (i.e., CRAB features) are
represented in green
Nursing Interventions: Myeloma
1. Pain management – NSAIDs can be very useful for mild pain or
can be given in combination with opioid analgesics
2. Needs to be educated about activity restrictions
- e.g., lifting no more than 10 pounds, the use of proper body
mechanics
3. Braces are occasionally needed to support the spinal column
4. Patients need to understand the importance of comprehensive
oral hygiene and good dental care
- to diminish the likelihood of developing osteonecrosis of
the jaw that may arise from bisphosphonate therapy
5. The patient also needs to be instructed about the s/s of
hypercalcemia
6. The patient needs to be instructed in appropriate infection
prevention measures and should be advised to contact the
primary provider immediately if fever or other s/s of infection
develop
7. It is important to maintain mobility and to use strategies that
enhance venous return (e.g., anti-embolism stockings, avoid
crossing the legs)
8. Nurse needs to carefully assess for symptoms related to
peripheral neuropathy and make assessments of the home for
safety
- Sensation (touch, temperature, pain, vibration,
proprioception), ankle reflexes, distal muscle strength, and
blood pressure should be evaluated
9. Patients should be educated to report any symptoms of
peripheral neuropathy and to not minimize such symptoms
- because prompt cessation of therapy or reducing the dose
can prevent the neuropathy from progressing.
BLOOD TYPING
- a method to tell what type of blood you have. Blood typing
is done so you can safely donate your blood or receive a
blood transfusion.
- It is also done to see if you have a substance called Rh factor
on the surface of your red blood cells
- Universal donor: O; Universal recipient: AB
BLOOD DONATION REQUIREMENTS
- Body weight should be at least 50kg (110lb) for a standard
450ml donation
- People younger than 17 years require parental consent in
some states
- The oral temperature should not exceed 37.5oC (99.6oF)
- The systolic arterial blood pressure should be 80 to 180 mm
HG, and the diastolic pressure should be 50 to 100mmHg
- The hemoglobin level should be at least 12.5 g/dL
Crossmatching
- a portion of donor blood is combined with patient plasma
or serum and is checked for agglutination, which would
signify incompatible blood
- This important step, also known as major crossmatch,
serves as the last guard to ensure a safe transfusion
Transfusion of Packed Red Blood Cells Pre-procedure
1. Confirm that the transfusion has been prescribed
2. Check that patient’s blood has been types and cross-matched
3. Verify that patient has signed a written consent form
4. Explain procedure to patient. Instruct patient in signs and
symptoms of transfusion reaction
5. Take patient’s temperature, pulse, respiration, blood pressure
and assess fluid volume status as a baseline for comparison
during transfusion.
6. Note if signs of increased fluid overload is present (e.g. heart
failure, contact hcp for prescription of diuretics)
7. Use hand hygiene and wear gloves in accordance with standard
precautions
8. Use appropriately sized needle for insertion in a peripheral vein.
Use special tubing that contains a blood filter to screen out fibrin
clots and other particulate matter. Do not vent blood container.
Transfusion of Packed Red Blood Cells Procedure
1. Obtain packed red blood cells (PRBCs) from the blood bank
after the IV line is started.
• Institution policy may limit release to only one
unit at a time.
2. Double check labels with another nurse or physician to
ensure that the ABO group and Rh type agree with the
compatibility record. Check to see that number and type on
 donor blood label and on patient’s medical record are
correct. Confirm patient’s identification by asking the
patient’s name and checking the identification wristband.
3. Check blood for gas bubbles and any unusual color or
cloudiness.
• Gas bubbles may indicate a bacterial growth.
Abnormal color or cloudiness may be signs of
hemolysis.
4. Make sure that PRBC transfusion is initiated within 30
minutes after removal of PRBCs from blood bank
refrigerator.
5. For the first 15 minutes, run the transfusion slowly – no
faster than 5mL/min. if no adverse effects occur during the
first 15 minutes, increase the flow rate unless patient is at
high risk for circulatory overload.
6. Monitor closely for 15-30 minutes to detect signs of
reaction. Monitor vital signs at regular intervals per
institution or agency policy; compare results with baseline
measurements. Observe patient frequently throughout the
transfusion for any signs of adverse reaction (restlessness,
hives, nausea, vomiting, torso or back pain, shortness of
breath, flushing, hematuria, fevers or chills). Should any
adverse reaction occur, stop infusion immediately, notify
primary provider and follow the agency’s transfusion
reaction standard.
7. Note the administration time does not exceed 4 hours
because of increased risk of bacterial proliferation.
8. Be alert for signs of adverse reactions: circulatory overload,
sepsis, febrile reaction, allergic reaction, and acute
hemolytic reaction.
9. Change blood tubing after every 2 units transfused to
decreases chance of bacterial contamination.
Transfusion of Packed Reb Blood Cells Post-procedure
1. Obtain vital signs and breath sounds; compare with
baseline measurements. If signs of increased fluid overload
present like heart failure, consider obtaining prescription
for diuretic.
2. Dispose of used materials properly
3. Document procedure in patient’s medical record including
patient assessment findings and tolerance to procedure.
4. Monitor patient for response to and effectiveness of
procedure. If patient is at risk, monitor for at least 6 hours
for signs of transfusion associated circulatory overload
(TACO); also monitor for signs of delayed hemolytic
reaction.
a. Take note: Never add medications to blood or
blood products. If blood is too thick to run freely,
you can put normal saline and if blood must be
warm, use an in-line blood warmer with the
monitoring system.
Transfusion of Platelets or Fresh-Frozen Plasma Pre procedure
1. Confirm that the transfusion has been prescribed.
2. Verify that the patient has signed a written consent form
and agrees to the procedure.
3. Explain procedure to patient. Instruct patient in signs and
symptoms of transfusion reaction (itching, hives, swelling,
shortness of breath, fever or chills).
4. Take patient’s temperature, pulse, respiration, blood
pressure and assess fluid status and auscultate breath
sounds to establish a baseline for comparison during
transfusion.
5. Note if signs of increased fluid overload present like heart
failure, contact primary provider to discuss potential need
for a prescription for diuretic as warranted. This is
particularly important when plasma is also infused.
6. Use hand hygiene and wear gloves in accordance with
standard precautions.
7. Use a 22-gauge or larger needle for placement in a large
vein, if possible. Use appropriate tubing for institution
policy.
Transfusion of Platelets or Fresh-frozen Plasma procedure
1. Obtain platelets or fresh-frozen plasma (FFP) from the
blood bank (only after the IV line is started).
2. Double check label with another nurse or physician to
ensure that the ABO group matches the compatibility
record (not unusually necessary for platelets; here only if
compatible platelets are ordered). Check to see that the
number and type on donor blood label and on patient’s
medical record are correct. Confirm patient’s identification
by asking the patient’s name and checking the identification
wrist band.
3. Check blood product for any unusual color or clumps
(excessive redness of the product indicates contamination
with larger amounts of red blood cells).
4. Make sure that platelets or FFP units are given immediately
after they are obtained.
5. Infuse each unit of FFP over 30-60 minutes per patient
tolerance; be prepared to infuse at a significant lower rate
in the context of fluid overload. Infuse each unit of platelets
as fast as patient can tolerate to diminish platelet clumping
during administration. Observe patient carefully for
adverse effects, especially circulatory overload. Decrease
rate of infusion if necessary.
6. Observe patient closely throughout transfusion for any
signs of adverse reaction (restlessness, hives, nausea,
vomiting, torso or back pain, shortness of breath, flushing,
hematuria, fevers or chills). Should any adverse reaction
occur, stop infusion immediately, notify primary provider
and follow the agency’s transfusion reaction standard.
7. Monitor vital signs at the end of transfusion per institution
policy; compare results with baseline measurements.
8. Flush line with saline after transfusion to remove blood
component from tubing.
Transfusion of Platelets or Fresh-Frozen Plasma Post-procedure
1. Obtain vital signs and auscultate breath sounds; compare
with baseline measurements. If signs of increased fluid
overload present like heart failure, consider obtaining
prescription for diuretic, as warranted
2. Dispose of used materials properly
3. Document procedure in patient’s medical record including
patient assessment findings and tolerance to procedure.
4. Monitor patient for response to and effectiveness of
procedure. A platelet count may be ordered 1 hour after
platelet transfusion to facilitate this evaluation.
5. If patient is at risk for transfusion-associated circulatory-
overload (TACO), monitor closely for 6 hours after
transfusion if possible.
a. Take note: FFT requires ABO but not Rh
compatibility. Platelets are not typically
crossmatch for ABO compatibility so never add
medications to blood or blood products.