Aplastic Anemia

APLASTIC ANEMIA HAZEL ARVEE B. POSIS

The bone marrow (soft tissue which is located within the hard outer shell of the bones) is responsible for the production of all the types of blood cells. The mature forms of these cells include red blood cells, which carry oxygen throughout the body; white blood cells, which fight infection; and platelets, which are involved in clotting. In aplastic anemia, the basic structure of the marrow becomes abnormal, and those cells responsible for generating blood cells (hematopoietic cells) are greatly decreased in number or absent. These hematopoietic cells are replaced by large quantities of fat.

 Is a physiological and anatomical failure of the bone marrow characterized by a marked decrease or absence of blood-forming elements in the marrow and peripheral pancytopenia (decreased RBCs ,WBCs and platelets ).  A rare and serious blood disorder in which bone marrow stops making enough new blood cells. aplastic anemia, normal production of all blood cells—red cells, white cells, and platelets—slows or stops. This is because the stem cells have been damaged. The cause of this damage is often unknown. Aplastic anemia is caused by damage to stem cells in the bone marrow. Stem cells normally develop into three types of blood cells: red blood cells, white blood cells, and platelets. When stem cells are damaged, they do not grow into healthy blood cells.

Causes Of Aplastic Anemia Inherited

Acquired

Fanconi anemia Familial aplastic anemia Dyskeratosis congenita Shwachman Diamond Syndrome Dubowitz syndrome

Idiopathic (70 % or more cases) Secondary • Drugs •6 –mecaptopurine •Methotrexate •Cyclophosphamide •Chloremphenicol Chemicals :insecticides Toxin(e.g.,benzen ,carbon tetrachloride) Irradiation Infection •Viral hepatitis •HIV •Infectious mononucleosis •CMV Myelodysplastic syndrome

CAUSES

DAMAGE TO STEM CELL

REPEATED INFECTION; FREQUENT SICK DAY

DEPRESSION OR CESSATION OF ACTIVITY OF ALL BLOOD PRODUCING ELEMENT

BLEEDING TENDENCY AEB ECCHYMOSIS, PURPURA, PETICHIAE, BLEEDING FROM NOSE, MOUTH, VAGINA, RECTUM

LEUKOPENIA THROMBOCYTOPENIA DECREASE IN FORMATION OF RBC

PANCYTOPENIA

APLASTIC ANEMIA

PALLOR OF SKIN & MUCOUS MEMBRANE, CYANOSIS

Clinical features of Aplastic Anemia 1. Anemia result in pallor ,easy fatigability ,weakness and loss of appetite 2. Thrombocytopenia leads to petechiae ,easy bruising ,severe nosebleeds and bleeding into the GIT and renal tract 3. Leukopenia leads to increased susceptibility to infections and oral ulcer that response poorly to antibiotic therapy. 4. Hepatosplenomegaly and lympadenopathy do not occur ; their presence suggest underlying leukemia 5. Hyperplastic gingivitis is also a symptom of aplastic anemia 6. Special features ; I. Skin ; Hyperpigmentation, café –au-lait spots,erythematous rash II. Head ;Microcephaly ,micro-ophthalmia III. Mouth ; cleft lip,leukoplakia IV. General : small stature

Nursing care is based on carefull assessment and mngt. Of the complication of pancytopenia

3. 4. 5. 6. 7. 8. 9.

Primary focused on preventing infection and monitoring for signs of bleeding To prevent infection in the hospitalized patient who is immunosupressed: Provision of private room Use of protective isolation Meticulous hygiene Protective oral care management. Monitoring of invasive lines for signs of infection Avoidance of bladder catheterization Instruct family and visitors of careful handwashing.

Monitor for signs of bleeding and provide measures to minimize risk Use a soft toothbrush and electric razor Avoid injections and venipuncture Hematest urine and stool Observe for oozing from gums, petechiae or ecchymoses Avoiding rectal thermometer, medications, enema. Monitor invasive lines sites.

Measures to avoid fatigue: Frequent periods of rest Avoid fatigue producing activities Monitor patient on signs of DOB.

O2 carrying of the blood O2 supply to tissues FATIGUE

Medical management 1. Blood transfusion: key to therapy until client’s own marrow begins to produce blood cells 2. Aggressive treatment of infections 3. Bone marrow transplantation 4. Drug therapy a. Corticosteroids and/or androgens to stimulate bone marrow function and to increase capillary resistance (Patients with aplastic anaemia are particularly vulnerable to damage to large joints which may result from steroid treatment)

1. Bone marrow aspiration, also called bone marrow sampling, is the removal by suction of fluid from the soft, spongy material that lines the inside of most bones. 2. Bone marrow biopsy, or needle biopsy, is the removal of a small piece of bone marrow. 3. A sample of the patient's bone marrow will need to be removed by needle (usually from the hip bone) and examined under a microscope. If aplastic anemia is present, this examination will reveal very few or no hematopoietic cells, and replacement with fat.

The most successful treatment for aplastic anemia is bone marrow transplantation. To do this, a marrow donor (often a sibling) must be identified. There are a number of tissue markers which must be examined to determine whether a bone marrow donation is likely to be compatible with the patient's immune system. Compatibility is necessary to avoid complications, including the destruction of the donor marrow by the patient's own immune system. Patients who cannot undergo bone marrow transplant can be treated with a number of agents, including • antithymocyte globulin (ATG) • cyclophosphamide • steroids •cyclosporine.

ANTITHYMOCYTE GLOBULIN An immunosuppressant which is used to reduce the body's natural immunity in patients who receive a bone marrow transplant or undergo treatment for Aplastic Anaemia It reduces the activity of the T lymphocytes which are attacking bone marrow stem cells. When a patient receives a bone marrow transplant , the white blood cells produced by the new stem cells in the bone marrow will try to get rid of (reject) the patients body. Antithymocyte globulin works by eliminating the white blood cells which are doing this. Before Having This Treatment allergic reaction to horses or rabbits Precautions: increase the chance of getting other infections fever, chills and shakes within a few hours after the first dose. Side Effects of This Medicine H20 retention Report: heart working harder, bloating or swelling

cyclophosphamide A drug used to treat cancers and to suppress the rejection of transplanted tissue. It basically kills off all the white cells . It is the drug of choice for those having a Bone Marrow Transplant. Cyclophosphamide commonly causes nausea, vomiting and hair loss and can affect the heart, lungs and liver. It can also cause severe bladder damage in susceptible people because it produces a toxic substance called acrolein (a colourless liquid with an irritating odour and used in chemical warfare as a tear gas). Another drug may be given before and after each dose of cyclophosphamide to reduce its toxicity. Inform your doctor if you find it painful to pass urine while you are on this drug. It can also lead to abnormal bleeding due to lowered blood cells, and increased risk of infection and reduced fertility in men. Drink lots of water while taking this drug. This will usually prevent it from causing bladder irritation.

STEROIDS One of a group of hormones chemically related to cholesterol. They include oestrogen, which controls female sexual development, androgen , which produces male characteristics and builds up muscular protein tissue, progesterone, which plays an important part in the regulation of the menstrual cycle and in pregnancy, and the corticosteroids , which are responsible for chemically making use of the carbohydrates and fats we eat, controlling inflammation, and salt and water regulation. Steroids may be naturally occurring or they may be synthesised. CYCLOSPORINE A drug used with ATG / ALG treatment to further suppress the immune system by inhibiting the activity of only the T lymphocytes . It is also used in bone marrow transplants to help handle GVHD . It also decreases the body's ability to fight off infections.

side effects confusion nervousness unusual weakness or tiredness difficulty breathing shortness of breath tender, enlarged, or bleeding gums nausea or vomiting stomach pain (severe) irregular heartbeat convulsions numbness when taking cyclosporine : 2. avoid eating excessive amounts of foods high in potassium such as bananas, oranges, orange juice, milk, waffles, oatmeal, tomatoes, and baked beans 2. avoid grapefruit juice as it may block the breakdown of cyclosporine by the liver (the opposite problem to the above). If this happens, blood levels of cyclosporine could be increased and this could increase serious side effects.