Aplastic anemia
<ul><li>What is aplastic anemia? </li></ul>
<ul><li>pancytopenia with hypocellularity (aplasia) of the bone marrow. </li></ul><ul><li>There are  no  leukaemic, cancer...
<ul><li>What is the underlying pathophysiology? </li></ul>
<ul><li>Due to a  reduction  in the number of  pluripotential stem cells  together with a fault in those remaining or  </l...
<ul><li>What are the causes? </li></ul>
 
<ul><li>What is the underlying immune mechanism that causes bone marrow failure? </li></ul>
<ul><li>Activated cytotoxic T cells  in blood and bone marrow are responsible for the bone marrow failure. </li></ul>
<ul><li>What are the drugs that cause marrow aplasia? </li></ul>
<ul><li>Cytotoxic drugs: </li></ul><ul><li>Busulfan </li></ul><ul><li>Doxorubicin </li></ul><ul><li>Non-cytotoxic drugs: <...
<ul><li>What is Fanconi’s anemia? </li></ul>
<ul><li>Fanconi’s anaemia is inherited as an autosomal recessive  </li></ul><ul><li>Associated with skeletal, renal and ce...
<ul><li>What are the clinical features of aplastic anemia? </li></ul>
<ul><li>Lack of Hb = Anemia  </li></ul><ul><li>Lack of platelet = Bleeding  </li></ul><ul><li>Lack of WCC = Infection </li...
<ul><li>What is the typical feature on marrow? </li></ul>
<ul><li>Pancytopenia </li></ul><ul><li>The virtual  absence of reticulocytes </li></ul><ul><li>A  hypocellular or aplastic...
 
<ul><li>What are the causes for pancytopenia? </li></ul>
 
<ul><li>What are the bad prognostic signs? </li></ul>
<ul><li>Presence of two of the following three features: </li></ul><ul><li>neutrophil count of < 0.5 × 109/L </li></ul><ul...
<ul><li>What is the treatment? </li></ul>
<ul><li>Supportive care- RBC/platelet transfusion prn </li></ul><ul><li>Treat infection early- Broad spectrum IV abx. </li...
<ul><li>What is the outcome of bone marrow transplant? </li></ul>
<ul><li>Results are poor for unrelated donors. </li></ul><ul><li>For those with HLA-identical sibling donor, 75–90% chance...
<ul><li>Why is bone marrow transplant not eligible for patients age >40? </li></ul>
<ul><li>High risk of  graft-versus-host disease  as a complication of bone marrow transplantation. </li></ul><ul><li>Use I...
<ul><li>Why is giving growth factors ineffective in aplastic anemia? </li></ul>
<ul><li>Levels of haemopoietic growth factors are normal or increased in most patients with aplastic anaemia </li></ul><ul...
<ul><li>What is the role of steroids? </li></ul>
<ul><li>Little activity in severe aplastic anaemia but are used for serum sickness due to ALG.  </li></ul><ul><li>They are...
<ul><li>What is the common condition associated with adult red cell aplasia? </li></ul>
<ul><li>Adult pure red cell aplasia is associated with a  thymoma  in 30% of cases and thymectomy may induce a remission. ...
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Aplastic anemia

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