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Aplastic anemia

Aplastic anemia






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    Aplastic anemia Aplastic anemia Presentation Transcript

    • Aplastic anemia
      • What is aplastic anemia?
      • pancytopenia with hypocellularity (aplasia) of the bone marrow.
      • There are no leukaemic, cancerous or other abnormal cells in the peripheral blood or bone marrow.
      • What is the underlying pathophysiology?
      • 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.
      • What are the causes?
      • What is the underlying immune mechanism that causes bone marrow failure?
      • Activated cytotoxic T cells in blood and bone marrow are responsible for the bone marrow failure.
      • What are the drugs that cause marrow aplasia?
      • Cytotoxic drugs:
      • Busulfan
      • Doxorubicin
      • Non-cytotoxic drugs:
      • chloramphenicol
      • gold
      • carbimazole
      • chlorpromazine
      • phenytoin
      • ribovirin
      • tolbutamide
      • NSAIDs.
      • What is Fanconi’s anemia?
      • 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.
      • What are the clinical features of aplastic anemia?
      • Lack of Hb = Anemia
      • Lack of platelet = Bleeding
      • Lack of WCC = Infection
      • What is the typical feature on marrow?
      • Pancytopenia
      • The virtual absence of reticulocytes
      • A hypocellular or aplastic bone marrow with increased fat spaces
      • What are the causes for pancytopenia?
      • What are the bad prognostic signs?
      • 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.
      • What is the treatment?
      • Supportive care- RBC/platelet transfusion prn
      • Treat infection early- Broad spectrum IV abx.
      • Bone marrow transplant ( Rx of Choice )-only if age <40.
      • What is the outcome of bone marrow transplant?
      • 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.
      • Why is bone marrow transplant not eligible for patients age >40?
      • 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%.
      • Why is giving growth factors ineffective in aplastic anemia?
      • Levels of haemopoietic growth factors are normal or increased in most patients with aplastic anaemia
      • Therefore they are ineffective as primary treatment.
      • What is the role of steroids?
      • 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).
      • What is the common condition associated with adult red cell aplasia?
      • Adult pure red cell aplasia is associated with a thymoma in 30% of cases and thymectomy may induce a remission.