| ID | 147 |
|---|---|
| Name | APLASTIC ANEMIA |
| Cause | |
| Signs Symptoms | |
| Diagnosis | |
| Investigations | B. Laboratory findings: Blood examination: Shows pancytopenia. There is no anisocytosis, poikilocytosis. No normoblast is found in the peripheral blood. M.C.H.C is normal. R.B.C. level is low, average 1.5 million/cmm. ‘W.B.C. level- 1000-2000/cmm. Polymorphs-10%, lymphocyte 90%. Platelet level is 10,000/cmm or less. Bone marrow examination: Reveals absence of red bone marrow and abundance of fat cells. |
| Management | |
| Introduction | Aplastic anemia is a consequence of bone marrow aplasia or failure that arises from injury to or abnormal expression of the stem cell, characterised by anemia, leucopenia and thrombocytopenia (i.e pancytopenia). |
| History | |
| Etiology | Etiology:1 1. Congenital (rare). 2. Idiopathic- probably autoimmune. 3. Systemic lupus erythematosus (SLE). 4. Chemotherapy, radiotherapy. 5. Drugs: Chloramphenicol, phenylbutazone, gold salts, sulfonamides, phenytoin, carbamazepine, quinacrine, tolbutamide. 6. Chemical poisons and toxins e.g Hair dyes, benzene, toluene, insecticides. 7. Infections- posthepatitis. 8. Pregnancy. 9. Paroxysmal nocturnal hemoglobinuria |
| Clinical Features | Clinical features: A. Symptoms and signs: Due to aplasia of the bone marrow, there will be anemia, leucopenia and thrombocytopenia. These three changes will produce varieties of symptoms and signs- 1. Due to anemia- there will be weakness, pallor, lassitude and others. 2. Due to leucopenia- there may be sore-throat, necrotic ulcer in the mouth, difficulty in swallowing, fever with chill and rigor and infections at different sites. 3. Due to thrombocytopenia- there is cutaneous and other forms of haemorrhage. |
| Preventions | |
| Treatment | Treatment: Mild aplastic anemia: 1. Supportive care and management 2. Red blood cell transfusions 3. Platelet transfusion (to control bleeding). 4. Proper antibiotic (broad spectrum) should be started immediately to prevent secondary infection. 5. The off ending drug (if any), should be stopped immediately. Severe aplastic anemia: 1. Bone marrow transplantation: Young adult patients (under age 50) who have HLA matched sibling donors, the treatment of choice is-allogeneic bone marrow transplantation. These patients may also be benefited from allogeneic transplantation using an unrelated donor (but the risk is more). 2. Immiuiosiippnsave therapy: Adult patients (over age 50) or those •without HLA-matched bone marrow donors, the treatment of choice is-immunosuppressive therapy with antithymocyte globulin (AGT) plus cyclosporine (or tacrolimus). ATG is given in the hospital in conjuction with transfusion and antibiotic support. A useful regimen is equine ATG 40mg/kg/day for 4 days in combination with cyclosporine 6mg/kg orally twice daily. (Rabbit ATG is more immunosuppressive than equine ATG and may be used in a dose of 3.5mg/kg twice daily for 4 days). Corticosteroid must be given in combination with ATG, such as-prednisolone l-2mg/kg/day initially, followed by a rapid taper, to avoid complications of serum sickness. Responses usually occur in 4-12 weeks and are usually only partial, but the blood counts rise high enough to give patients a safe and transfusion-free life. High dose immunosuppression with cyclophosphamide 200mg/kg, has produced remission in refractory cases and should be considered for pateints without suitable bone marrow donors. 3. One regimen of oxymetholone (bone marrow stimulating agent) 2-3mg/kg orally daily, may be given up to 3-6 months. 4. Splenectomy is said to be beneficial in some cases. |
| Complications | |
| Prognosis | |
| Types | Types of aplastic anemia: On laboratory findings aplastic anemia may be divided in to- i. Mild aplastic anemia and ii. Severe aplastic anemia. Aplastic anemia is said to be severe when- - neutrophil count < 500/mcl, - platelet count < 20,000/mcl, - reticulocyte count < 1%, and - bone marrow cellularity < 20%. When this picture is present (or three of the four), the median survival without treatment is approximately 3 months, and only 20% of patients survise for 1 year. Investigations: See above under the laboratory findings. |
| Classification | |
| Observation | |
| Pathology |
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