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aplastic_anaemia

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aplastic anaemia / pancytopenia

Introduction

  • aplastic anaemia is an auto-immune, rare (1-4 per million population) life threatening disorder in which the stem cells in bone marrow are destroyed leading to severe anaemia, thrombocytopenia and agranulocytosis (ie. pancytopenia)
  • more frequent in teenagers and young adult but is also common among the elderly
  • many have clones of cells (perhaps as an escape mechanism from immune destruction of stem cells) characteristic of paroxysmal nocturnal haemoglobinuria
  • Rx is generally immunosuppressive drugs, blood transfusions and protection from infections whilst awaiting bone marrow transplant
    • for allografting (grafting between individuals), the donor's stem cells must be as compatible as possible with those of the recipient to avoid the risk of immunological complications including graft-versus-host disease

Aetiology

  • ~50% of cases are idiopathic
  • post-viral
  • vaccines
  • medications
    • cytotoxic chemotherapy
    • chloramphenicol (> 1 in 40,000 Rx courses perhaps 1 in 100,000 risk?)
    • carbamazepine
    • felbamate
    • phenytoin
    • quinine
    • phenylbutazone
  • chemicals
    • benzene
  • radiation

Prognosis

  • mild disease may self-resolve
  • severe disease:
    • high risk of death if untreated
    • with stem cell transplant, 5 yr survival rates are1):
      • 42% for age < 20yrs
      • 32% for 20-40yrs
      • 10% for age > 40yrs
    • elderly not suited to stem cell transplants have a 5 yr survival up to 35% with immunosuppressive Rx
  • 10–15% of severe aplastic anemia cases evolve into myelodysplastic syndrome and leukemia
aplastic_anaemia.txt · Last modified: 2024/03/24 13:21 by gary1

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