Aplastic Anemia

I. Definition: Aplastic anemia is a condition where bone marrow does not produce sufficient new cells to replenish blood cells. The condition, as the name indicates, involves both aplasia and anemia. Typically, anemia refers to low red blood cell counts, but aplastic anemia patients have lower counts of all three blood cell types: red blood cells, white blood cells, and platelets, termed pancytopenia.

II. Anatomy and Physiology:

Bone Marrow

Bone marrow is the flexible tissue found in the interior of bones. In humans, red blood cells are produced in the heads of long bones, in a process known as hematopoesis. On average, bone marrow constitutes 4% of the total body mass of humans; in an adult weighing 65 kilograms (140 lb), bone marrow accounts for approximately 2.6 kilograms (5.7 lb). The hematopoietic compartment of bone marrow produces approximately 500 billion blood cells per day, which use the bone marrow vasculature as a conduit to the body's systemic circulation.[1] Bone marrow is also a key component of the lymphatic system, producing the lymphocytes that support the body's immune system.[2]

Marrow Types The two types of bone marrow are medulla ossium rubra (red marrow), which consists mainly of hematopoietic tissue, and medulla ossium flava (yellow marrow), which is mainly made up of fat cells. Red blood cells, platelets and most white blood cells arise in red marrow. Both types of bone marrow contain numerous blood vessels and capillaries. At birth, all bone marrow is red. With age, more and more of it is converted to the yellow type; only around half of adult bone marrow is red. Red marrow is found mainly in the flat bones, such as the pelvis, sternum, cranium, ribs, vertebrae and scapulae, and in the cancellous ("spongy") material at the epiphyseal ends oflong bones such as the femur and humerus. Yellow marrow is found in the medullary cavity, the hollow interior of the middle portion of long bones. In cases of severe blood loss, the body can convert yellow marrow back to red marrow to increase blood cell production. Stroma The stroma of the bone marrow is all tissue not directly involved in the primary function of hematopoiesis. Yellow bone marrow makes up the majority of bone marrow stroma, in addition to smaller concentrations of stromal cells located in the red bone marrow. Though not as active as parenchymal red marrow, stroma is indirectly involved in hematopoiesis, since it provides the hematopoietic microenvironment that facilitates hematopoiesis by the parenchymal cells. For instance, they generate colony stimulating factors, which have a significant effect on hematopoiesis. Cells that constitute the bone marrow stroma are:

fibroblasts (reticular connective tissue) macrophages adipocytes osteoblasts osteoclasts endothelial cells, which form the sinusoids. These derive from endothelial stem cells, which are also present in the bone marrow.[3] to red blood cell production, as they

Macrophages contribute especially deliver iron for hemoglobin production.

Bone Marrow Barrier

The blood vessels of the bone marrow constitute a barrier, inhibiting immature blood cells from leaving the marrow. Only mature blood cells contain the membrane proteins required to attach to and pass the blood vessel endothelium. Hematopoietic stem cells may also cross the bone marrow barrier, and may thus be harvested from blood. III. Etiology and Epidemiology

Aplastic anemia affects all age groups and both genders. In approximately half of patients with aplastic anemia in North America, no etiologic agent is identifiable. Identified

causes of aplastic anemia include antineoplastic drugs and exposure to certain drugs, including chloramphenicol, sulfonamides, and anticonvulsant agents such as phenytoin ( Dilantin). Insecticides such as chlorophenothane and chemicals, particularly benzene, also are thought to cause aplastic anemia. Infections associated with the pathogenesis of aplastic anemia include hepatitis types B and C, Epstein-barr virus infection, cytomegalovirus infection, and military tuberculosis. The defect leading to aplastic anemia is most likely injury or populations. Aplastic anemia may also be congenital. IV. Symptomatology In the majority of cases, the onset is insidious. Because the entire bone marrow is affected, manifestations include those of: -anemia (pallor, weakness, and dyspnea); - leucopenia, such as recurrent or multiple infections; -thrombocytopenia (petechiae-flat, red, pinpoint hemorrhages on the skin and a tendency to bleed excessively, particularly in the mouth)

V. Pathophysiology

VI. Medical Management Laboratory Test -CBC: complete blood count determines the blood component count and gauges if it is within the normal range -Bone Marrow Biopsy: examines the source of the blood cells in the bone marrow to obtain more information on hematopoiesis Treatment Prompt treatment of the underlying cause and removal of any bone marrow suppressants are essential to recovery of the bone marrow. Blood transfusion may be necessary if stem cell levels are very low. Bone marrow transplantation may be helpful in younger patients; its success depends on the accuracy of the tissue match using human leukocyte antigen (HLA). Chemotherapy and radiation are used to prepare the recipient;s bone marrow for transplantation of stem cells (taken from the marrow of the pelvic bone of a suitable donor). Newer techniques allow harvesting of stem cells from the peripheral blood, not the marrow. The donor of stem cells are infused intravenously into the blood of the recipient; they migrate to the bone marrow and provide a new source of blood cells after several weeks. Antirejection drugs are required for a year.

VII. Nursing Diagnosis

Activity Intolerance r/t weakness, fatigue.

Imbalanced Nutrition- less than body requirement r/t decreased knowledge of nutritional needs. Deficient Knowledge with regards to balanced diet Risk for injury Risk for bleeding

VIII. Nursing management

Monitor vital signs, especially temp. Stress proper hand washing techniques by all caregivers between therapies Monitor visitors/ caregivers of the client. Instruct SO to limit visitors especially those with known contagious illnesses. Maintain aseptic/sterile techniques as much as possible Advise the use of facial mask when going outside the clients room

Encouraged early ambulation, deep breathing and turning exercises as indicated. Provide regular catheter/ perineal care as indicated Reinforce teachings about diet. Avoid raw meats, fruits and vegetables. Consume prescribed nutritionally adequate menus. Enforce strict bed rest. Provide the client her favorite books as necessary. Review to the client and SO about the nature of the disease and the interventions needed.