aplastic_anaemia
Table of Contents
aplastic anaemia / pancytopenia
see also:
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
- 2% of cases of acute viral hepatitis
- parvovirus may cause a short-lived aplastic anaemia as parvovirus binds to P antigen and causes temporary cessation of red cell production which is usually unnoticed as RBC life span is 120 days
- 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