Download - Aplastic anemia
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Aplastic anemia
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• What is aplastic anemia?
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• pancytopenia with hypocellularity (aplasia) of the bone marrow.
• There are no leukaemic, cancerous or other abnormal cells in the peripheral blood or bone marrow.
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• What is the underlying pathophysiology?
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• Due to a reduction in the number of pluripotential stem cells together with a fault in those remaining or
• An immune reaction against them so that they are unable to repopulate the bone marrow.
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• What are the causes?
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• What is the underlying immune mechanism that causes bone marrow failure?
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• Activated cytotoxic T cells in blood and bone marrow are responsible for the bone marrow failure.
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• What are the drugs that cause marrow aplasia?
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Cytotoxic drugs:• Busulfan• Doxorubicin
Non-cytotoxic drugs:• chloramphenicol• gold• carbimazole• chlorpromazine• phenytoin• ribovirin• tolbutamide• NSAIDs.
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• What is Fanconi’s anemia?
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• Fanconi’s anaemia is inherited as an autosomal recessive
• Associated with skeletal, renal and central nervous system abnormalities.
• It usually presents between the ages of 5 and 10 years.
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• What are the clinical features of aplastic anemia?
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• Lack of Hb = Anemia • Lack of platelet = Bleeding • Lack of WCC = Infection
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• What is the typical feature on marrow?
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• Pancytopenia• The virtual absence of reticulocytes• A hypocellular or aplastic bone marrow with
increased fat spaces
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• What are the causes for pancytopenia?
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• What are the bad prognostic signs?
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Presence of two of the following three features:• neutrophil count of < 0.5 × 109/L• platelet count of < 20 × 109/L• reticulocyte count of < 40 × 109/L.
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• What is the treatment?
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• Supportive care- RBC/platelet transfusion prn• Treat infection early- Broad spectrum IV abx.• Bone marrow transplant (Rx of Choice)-only if
age <40.
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• What is the outcome of bone marrow transplant?
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• Results are poor for unrelated donors.
• For those with HLA-identical sibling donor, 75–90% chance of long-term survival and restoring the blood count to normal.
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• Why is bone marrow transplant not eligible for patients age >40?
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• High risk of graft-versus-host disease as a complication of bone marrow transplantation.
• Use Immunosuppressive therapy, antilymphocyte globulin (ALG) and ciclosporin in combination which gives a response rate of 60–80%.
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• Why is giving growth factors ineffective in aplastic anemia?
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• Levels of haemopoietic growth factors are normal or increased in most patients with aplastic anaemia
• Therefore they are ineffective as primary treatment.
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• What is the role of steroids?
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• Little activity in severe aplastic anaemia but are used for serum sickness due to ALG.
• They are also used to treat children with congenital pure red cell aplasia (Diamond–Blackfan syndrome).
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• What is the common condition associated with adult red cell aplasia?
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• Adult pure red cell aplasia is associated with a thymoma in 30% of cases and thymectomy may induce a remission.