Aplastic anemia can be acquired or inherited. Acquired forms can be caused by drugs (chloramphenicol), chemicals (benzene), radiation, or viral infection (hepatitis, Epstein-Barr) and, in rare instances, are associated with paroxysmal nocturnal hemoglobinuria. Fanconi's anemia is the most common inherited type. Prognosis is grave. Fifty percent of patients die within 6 months of diagnosis.
Aplastic anemia may occur at any age. Fifty percent of cases are idiopathic. Long-term survival rate with bone marrow transplant (BMT) from histocompatible donors is as high as 70% to 90% in children.
Aplastic anemia is a syndrome of bone-marrow failure characterized by peripheral pancytopenia and marrow hypoplasia.
The manifestation of symptoms is dependent on the extent of the thrombocytopenia (hemorrhagic symptoms), neutropenia (bacterial infections, fever), and anemia (pallor, fatigue, congestive heart failure, tachycardia). Severe aplastic anemia is characterized by a granulocyte count of less than 500 per mm3, a platelet count of less than 20,000 per mm3, and a reticulocyte count of less than 1.
Clinical manifestations include petechiae, ecchymoses, epistaxis (occur first); oral ulcerations, bacterial infections, fever (occur later in course); anemia, pallor, fatigue, tachycardia (late signs); and cafe au lait spots, melanin-like hyperpigmentation, absent thumbs (Fanconi's anemia).
Complete blood count with differential—macrocytic anemia; decreased granulocytes, monocytes, and lymphocytes
Bone marrow aspiration and biopsy--hypocellular
Hemoglobin electrophoresis---elevated fetal hemoglobin level
Red cell i antigen titer--elevated
Sugar water test--positive
Serum folate and B12 levels--normal or elevated
Chromosome breakage test--positive for Fanconi's anemia
The first-choice treatment for aplastic anemia is bone marrow transplant with a sibling donor who is human lymphocyte antigens (HLA) matched. In more than 70% of cases there will be no sibling match. However, there is an increased chance that there will be a match between one parent and the child with aplastic anemia. If bone marrow transplant is to be done, HLA typing of the family is done immediately and blood products are used as little as possible to avoid sensitization. Also to avoid sensitization, blood should not be donated by the child's family. Blood products should always be irradiated and filtered to remove white blood cells before being given to a child who is a candidate for bone marrow transplant.
Immunotherapy with either antithymocyte globulin (ATG) or antilymphocyte globulin (ALG) is the primary treatment for those children not candidates for bone marrow transplant. The child will respond within 3 months or not at all to this therapy. Cyclosporine A is also an effective immunosuppresant that can be used in the treatment of aplastic anemia. Androgens are rarely used unless no other treatment is available.
Supportive therapy includes use of antibiotics and administration of blood products. Antibiotics are used to treat fever and neutropenia; prophylactic antibiotics are not indicated for the asymptomatic child. Blood product administration may include the following:
1. Platelets--to maintain platelet count greater than 20,000 per mm3. Use single-donor platelet pheresis to decrease number of human lymphocyte antigens to which the child is exposed.
2. Packed red blood cells--to maintain hemoglobin (Hb) level higher than g/dl (chronic anemia is often well tolerated). For long-term therapy, use deferoxamine as chelating agent to prevent complications of iron overload.
3. Granulocytes--to transfuse to patient who has gram negative sepsis.
Child will have gradual increase in red blood cells, white blood cells, and eventually platelets.
Child will have fewer infections.
Child will have minimal bleeding episodes.
Child and family understand home care and follow-up needs.
Risk for impaired oral mucous membrane related to tissue hypoxia and vulnerability
Risk for infection related to increased susceptibility secondary to leukopenia
Activity intolerance related to insufficient oxygen secondary to diminished red blood cell count
Risk for ineffective therapeutic regimen management related to insufficient knowledge of causes, prevention, and signs and symptoms of complications
Identify and report signs and symptoms of hemorrhage.
Vital signs (increased apical pulse, thready pulse, decreased blood pressure)
Skin color (pallor) and signs of diaphoresis
Decreased level of consciousness
Decreased platelet count
Protect from trauma.
Do not administer aspirin or nonsteroidal antiinflammatory drugs (NSAIDs)
Avoid use of intramuscular (IM) injection and suppositories.
Administer contraceptive to decrease excessive menstruation.
Provide good oral hygiene with soft toothbrush.
Protect from infection.
Limit contact with potential source of infection.
Use strict isolation precautions (refer to institution's policies and procedures).
Administer blood products and monitor child's response to their infusion.
Observe for side effects or untoward response (transfusion reaction).
Observe for signs of fluid overload.
Monitor vital signs before infusion; monitor every 15 minutes during first hour and then hourly during infusion.
Provide frequent rest periods. Organize nursing care to increase activity tolerance and prevent fatigue.
Monitor child's therapeutic and untoward response to medications; monitor the action and side effects of administered medications.
Prepare child and family for bone marrow transplant.
Monitor for signs of bone marrow transplant complications.
Provide age-appropriate diversional and recreational activities.
Provide age-appropriate explanation before procedures.
Instruct parents about measures to protect child from infection.
Limit contact with infectious agents.
Identify signs and symptoms of infection.
Instruct parents to monitor for signs of complications.
Instruct parents about the administration of medication.
Monitor child's therapeutic response.
Monitor for untoward responses.
Provide child and family with information about community support systems for long-term adaptation.
Child and sibling groups
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