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Blood cells are produce in the bones of the body mainly in the bone of the pelvis, ribs, sternum through A
process called hematopoiesis. This process starts in the bone marrow, the innermost portion of the bone
where the hematopoietic stem cells resided. These serve as the progenitor cells for all the different cells
type found in the blood.

In the process hematopoiesis also called as hemocytoblasts can become a lymphoid progenitors or
myeloid progenitors. The lymphoid progenitors can develop into lymphoblast which can then differentiate
to some white blood cells such as t-lymphocytes, lymphocytes, and natural killer cells. Myeloid
progenitors can be differentiate into erythrocytes or red blood cells, megakaryocytes which eventually
give rise to platelets or myeloblasts which can then be the other white blood cells like monocytes,
neutrophils, basophils, and eosinophils

Normal values
RED BLOOD CELLS - 4.2–6.2 million
PLATELETS - 250-400 thousands
WHITE BLOOD CELLS - 5-10 thousands
 NEUTROPHILS - 60%-70%
 LYMPHOCYTES - 20%-25%
 MONOCYTES - 3%-8%
 EOSINOPHILS - 2%-4%
 BASOPHILS - 0.5%-1%

What happen in this aplastic anemia is when there is damage to hematopoietic stem cells. This may
result to decrease production in RBC, decrease production in platelets, and decrease production in WBC.
Decrease RBC also termed as anemia, decrease platelet as thrombocytopenia, and decrease WBC as
leukopenia. These three conditions may called as pancytopenia. From the word itself we can understand
that pan means all, cyto means blood cells, and penia means decrease in number. So pancytopenia is
the decrease of all types of blood cells

In most cases, the etiology of aplastic anemia is idiopathic or without apparent cause. Definable
cause of aplastic anemia include autoimmune disease, genetic disorder such as fanconi anemia, Viral
infections such as parovirus, hepatitis, and epstein barr virus, Drugs such as chemotherapeutic agents,
anti-seizure, anti-inflammatory, antithyroid, and antibiotic like sulfonamides and chloramphenicol can
destroy hematopoietic stem cells, chemicals and toxins that may produce marrow aplasia such as
benzene and benzene derivatives that may found in airplane glue, paint remover, dry-cleaning solution.
Certain toxic materials, such as inorganic arsenic, glycol ethers, plutonium, and radon, have also been
implicated as potential causes. Lastly, exposure to radiation

The clinical manifestations for anemia are pallor, fatigue, dyspnea, and palpitation. For thrombocytopenia
are epistaxis, purpura, petechiae, and retinal damage. For neutropenia is recurrent infection. It also
manifest such as fever, headache, lymphadenopathy and splenomegaly, pain, erythema, and body
malaise.

For diagnostic and laboratory procedure, there is CBC or complete blood count which may reveal
decrease RBC, platelet and WBC. Another is bone marrow aspiration and biopsy which may show
hypocellularity, decrease progenitor cells, and increase fat cells.

For medical management, any offending agents are discontinued or remove such as medication,
chemicals, radiation, and viral infection. Another treatment is immunosuppressive therapy.
Immunosuppressants prevent the patient’s lymphocytes from destroying the stem cells. This includes
cyclosporine, ATG, glucocorticoids, and granulocyte colony stimulating factor. For severe cases, it is
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recommended to have a blood transfusion. Another treatment is bone marrow transplantation or

peripheral blood stem cell transplantation.

For nursing Management, Patients with aplastic anemia are vulnerable to problems related to erythrocyte,
leukocyte, and platelet insufficiencies. They should be assessed carefully for signs of infection and
bleeding.

To minimize risk of infection:

▪ Implement reverse/protective isolation. Provide
private room; practice strict handwashing
▪ Encourage good personal hygiene including good
oral care daily shower or bath with mild soap, and
perirectal care after using the toilet.
▪ Monitor vital signs including temperature
frequently; notify health provider of fever.
▪ ▪ Minimize invasive procedures or possible trauma to
skin or mucous membranes

To minimize risk of bleeding:

▪ Use soft toothbrush or toothettes for mouth care;
electric razor for shaving, keep nails short by filing.
▪ Avoid intramuscular injections and other invasive
procedures.
▪ Prevent constipation by use of stool softeners
(Colace/Docussate Na) as prescribed.
▪ Restrict activity based on platelet count and active
bleeding
▪ Monitor pad count for menstruating patient; avoid
use of vaginal tampons
▪ Control bleeding by applying pressure to the site,
using ice packs and prescribed topical hemostatic
agents

Nurses must also monitor for side effects of therapy, particularly for hypersensitivity reaction while
administering ATG. If patients require long-term cyclosporine therapy, they should be monitored for long-
term effects, including renal or liver dysfunction, hypertension, pruritus, visual impairment, tremor, and
skin cancer. They should also be informed that the metabolism of ATG is altered by many other
medications; thus, each new prescription needs careful assessment for drug–drug interactions.

The following are the nursing diagnosis

The prognosis for idiopathic aplasia lies between these 2 extremes, with an untreated mortality rate of
approximately 60-70% within 2 years after diagnosis. The 2-year fatality rate for severe aplastic anemia is
70% without bone marrow transplantation or a response to immunosuppressive therapy
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In megaloblastic anemia, there is an impaired DNA synthesis which results in unusually large,
structurally abnormal, immature red blood cells. Other cells derived from the myeloid stem cell
(nonlymphoid leukocytes, platelets) are also abnormal. A bone marrow analysis reveals hyperplasia (an
abnormal increase in the number of cells), and the precursor erythroid and myeloid cells are large and
bizarre in appearance. However, many of these abnormal erythroid and myeloid cells are destroyed
within the marrow, so the mature cells that do leave the marrow are actually fewer in number. Thus,
pancytopenia can develop. Because the erythrocytes are very large, the MCV is very high, usually
exceeding 110 fl.

There are two main causes, the Vitamin B12 deficiency or the folic acid deficiency. Vitamin B12 and folic
acid are essential for normal DNA synthesis.

Folic acid is stored as compounds referred to as folates. The folate stores in the body are much smaller
than those of vitamin B12 and can become depleted within months when the dietary intake of folate is
deficient (Green, 2016). Folate is found in green vegetables and liver.

We eat folate in the form of polyglutamate. These polyglutamate can be absorbed at the small intestine
particularly in jejunum. These polyglutamate can be converted to monoglutamate by the enzyme
conjugase. These monoglutamate ie folate will go into the blood and it goes into the bloodstream in the
form of methyl tetrahydrofolate. For tetrahydrofolate to participate in DNA synthesis it has to get rid of
methyl group. So it shifts the ethyl group and give it to the B12, now the cobalamin is methyl cobalamin.
The tetrahydrofolate is free and it can participate in the DNA synthesis. Tetrahydrofolate can be
converted into methylene tetrahydrofolate and will be converted into dihydrofolate which can be
converted back to the tetrahydrofolate by dihydrofolate reductase to complete the cycle. And at the
same step the d UMP is converted by thymidylate synthase into d TMP and this d TMP is part of the
DNA. Here in the methyl cobalamin, in order for it to participate in DNA synthesis, it has to get rid of that
methyl group so it give it to homocysteine. Homocysteine plus methyl equals methionine. This process
can be done by the help of the enzyme homocysteine synthase

Folate deficiency occurs in people who rarely eat uncooked vegetables. Another one is chronic
alcoholism. Alcohol increases folic acid requirements, and, at the same time, patients with alcoholism
usually have a diet that is deficient in the vitamin. Some patients with malabsorptive diseases of the
small bowel, such as celiac disease, may not absorb folic acid normally. Folic acid requirements are also
increased in patients with liver disease, chronic hemolytic anemias and in women who are pregnant,
because the need for erythrocyte production is increased in these conditions.

So when folate deficiency occurs the Tetrahydrofolate levels are low which may lead to slowed DNA
replication. In RBCs, slower DNA replication means fewer cell mitosis occur before cell matures. Less cell
divisions means fewer, but larger RBCs. This may result to megaloblastic anemia.

The clinical manifestations are cracked lips, sore tongue, decrease RBC, hemoglocin and hematocrit,
increase MCV, palpitations, fatigue and weakness, pallor and slightly jaundice, forgetfulness + fainting +
irritability, and nausea and anorexia.
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For diagnostic test, there should be blood studies, schilling test, and intrinsic factor antibody test.

For Medical Management Folate deficiency is treated by increasing the amount of folic acid in the diet
and administering 1 mg of folic acid daily. Folic acid is given intramuscularly only to people with
malabsorption problems. Although many multivitamin preparations now contain folic acid, additional
supplements may be necessary because the amount may be inadequate to fully replace deficient body
stores. Patients who abuse alcohol should receive folic acid as long as they continue to consume alcohol.

Another cause of megaloblastic anemia is Vitamin B12 deficiency. Vitamin B12 also known as cobalamin
is essential to Dna synthesis, nuclear maturation, and healthy myelin. Vitamin B12 can be found in meat
and dairy products.

A deficiency of vitamin B12 can occur in several ways. Inadequate dietary intake is rare but can
develop in strict vegans who consume no meat or dairy products. It can also occurs when patients
undergo or has an history of gastric surgery. Decreased B12 absorption also occurs in conditions such as
Crohn’s disease. Faulty absorption from the GI tract is more common, particularly in the older adult.).
Another cause is autoimmune antibody. The autoimmune reaction results in atrophy of the parietal
cells, leading to achlorhydria and lack of the glycoprotein intrinsic factor. The absence of intrinsic factor;
in this particular context, the resultant anemia is called pernicious anemia. Decreased intrinsic factor
production by the parietal cells of the stomach causes decreased Vit B12 absorption. Vitamin B12 has to
bind with intrinsic factor in the stomach so that it can be absorbed in the terminal ileum of the small
intestines by cells that have receptors for the B12 IF complex. Once it is absorbed, it travels to the liver,
where it is stored. A vitamin B12 deficiency does not become apparent for approximately 3 years after
absorption ceases because the liver stores 2,000 to 5,000 µg of Vit b12 and daily losses are only 3-
5µg/day. Decreased Vitamin B12 absorption results in the following: Decreased RBC production,
Decreased DNA synthesis in maturing RBCs; the RBCs do not divide normally so they grow big resulting
to megalobastic cells, and Impaired integrity of the cells in the GI tract (mouth, stomach and anus),
vagina and axon of neurons.

Vitamiin B12 deficiency may lead to disruption in DNA synthesis, to systemic manifestation . This may
result to megaloblastic anemia.

The clinical manifestations are neurological syndrome, paresthesia of the hands and feet, movement
disorders, neuropsychiatric changes, bladder and bowel dysfunction, beefy red, inflamed tongue,
fatigue,jaundice, anisocytosis, poikilocytosis, hyperpigmented neutrophils, and low V B12 serum level

For diagnostic test, there should be blood studies, schilling test, and intrinsic factor antibody test, and
bone marrow aspiration

For medical management , Vitamin B12 deficiency is treated by vitamin B12 replacement. Vegans can
prevent or treat deficiency with oral supplements with vitamins or fortified soy milk. When the
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deficiency is due to the more common defect in absorption or the absence of intrinsic factor,
replacement is by monthly intramuscular injections of vitamin B12.

The following are the nursing diagnosis

The prognosis is favorable if the etiology of megaloblastosis has been identified and appropriate
treatment has been instituted. However, patients are at risk for hypokalemia and anemia-related cardiac
complications during therapy for cobalamin deficiency

Myelodysplastic syndrome (MDS) is a group of related hematologic disorders characterized by

peripheral blood cytopenias (from ineffective blood cell production) and changes in the cellularity of the
bone marrow with dysplastic changes. In MDS, hematopoiesis is disorderly and ineffective. It affects
individual of all ages, more common after the age of 60.

As discuss earlier, blood cells develop from the hematopoietic stem cells through a process called
hematopoiesis. This involves a number of divisions and eventually results in three types of blood cells
such as RBC, white blood cells, and platelets. Once mature, it leave the bone marrow and enter the
blood steam. In MDS, hematopoietic stem cells are damaged so they give rise to faulty blood cells which
don’t mature but instead persist as immature cells called blast. These immature cells are usually die in
the bone marrow or soon after they go into the blood so you cant really count on them to do the job of
mature cells. as the condition progresses, immature blood cells gradually take over the bone marrow
which displaces and reduces the normal ones.

In most cases, the causes of myelodysplastic syndrome is unknown or idiopathic. When this happens it is
classified as primary MDS. In rare cases, they can be cause by prior exposure to chemicals, including
benzene, radiation, and chemotherapeutic medications (particularly alkylating agents and
topoisomerase inhibitors). When this happen it is classified as secondary MDS. Secondary MDS is less
common (10%), but has a poorer prognosis than does primary MDS, as it tends to be resistant to
treatment, has more cytogenetic abnormalities associated with it, and evolves into AML more
frequently. Secondary MDS can occur at any age

The manifestations of MDS can vary widely. Some patients are asymptomatic, with the illness
being discovered incidentally when a CBC is performed for other purposes. Other patients have
profound symptoms and complications from the illness. Because MDS tends to occur in older adults,
other concurrent chronic health conditions may exacerbate symptoms associated with the disease.
Fatigue is often present, with varying levels of intensity and frequency. Neutrophil dysfunction puts the
person at risk for recurrent pneumonias and other infections. Because platelet function can also be
altered, bleeding can occur. These problems may persist in a fairly steady state for months, even years.
Over time, the marrow may fail to provide enough cells despite support with transfusion or growth
factors; this is called bone marrow failure. MDS may also progress over time; as the dysplasia evolves
into a leukemic state, so leukemic manifestation may also present
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The diagnosis of MDS requires laboratory testing, which may include:

typically reveals a macrocytic anemia; leukocyte and platelet

●A complete blood count which
counts may be diminished as well.
●A blood smear – to determine the number, size, shape, maturity, and type of blood cells and
whether they look normal.
● The 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), and Cytogenetic
analysis is important in determining the overall prognosis, risk of evolution into AML, and
method of treatment

Management Medical management strategies are based on the stage of disease and prognosis. The goal
for treating patients with low-risk disease is to improve cytopenias, decrease blood transfusion
requirements, and improve quality of life. The goal for treating patients with high-risk disease is to
decrease the likelihood that the disease transforms into AML and to extend survival.

Stem cell transplantation also called hematopoietic stem cell transplantation or bone marrow
transplantation) is the treatment for MDS most associated with long-term survival, although long-term
survivors can have complications such as "graft-versus-host disease" (GVHD). As with all therapies, the
potential risks of transplant must be considered together with the potential benefits relative to other
treatment options. 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). Transfusions of red blood cells may be needed to treat symptoms of anemia,
including fatigue or shortness of breath. Transfusions of red blood cells can relieve symptoms, but if many
are given (usually more than 30 transfusions), an accumulation of iron can cause organ damage ("iron
overload") and may require special treatment to remove the excess iron (called "iron chelation" or
chelation therapy). Transfusions of platelets can prevent or treat bleeding problems caused by having too
few platelets. Platelets survive for only about a few days, so platelet transfusions may be needed more
frequently. Attempts to improve anemia and decrease RBC transfusion are often successful with the use
of erythroid-stimulating agents (epoetin alfa [Procrit] or darbopoetin alfa [Aranesp]). Higher-than-
normal doses may be required to achieve an adequate improvement in hemoglobin. Adding myeloid
growth factors such as granulocyte colony-stimulating factor G-CSF can boost responsiveness to these
agents. This stimulates white blood cell (granulocyte) production, and may raise the white blood cell
count. G-CSF is generally only used in the setting of a severe infection.

Caring for patients with MDS can be challenging because the illness is unpredictable. As with other
hematologic disorders, some patients (especially those with no symptoms) have difficulty perceiving
that they have a serious illness that can place them at risk of life-threatening complications. It is
imperative that the nurse recognizes any concurrent health problems the patient may have. This
knowledge will help the nurse better plan and manage the patient’s care.

For nursing management, extensive instruction on infection and bleeding risks, monitoring of laboratory
values, anticipate the need for transfusion, and instruct about receiving chelation therapy.
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The nursing diagnosis are the following

For people who are diagnosed with MDS, the estimated length of survival is influenced by the risk
category, the presence of underlying medical problems, and age. However, these numbers represent
averages, and do not necessarily predict what will happen in your situation. There is considerable
variation from person to person, especially in the lower-risk group.

Survival statistics for MDS.

WPSS Risk Group Median Survival Risk of AML (within 5 years)

Low 5.5 years 14%

Intermediate 4 years 33%

High 2.2 years 54%

Very high 9 months 84%