Canterbury DHB
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Aplastic Anaemia (AA) is a rare and often fatal disorder with an incidence of 2 to 3 million per year.
It may be congenital or acquired, and occasionally the congenital variety (Fanconi Anaemia) may not present until the second or third decade.
AA often requires prompt diagnosis and treatment, and in younger patients (under 40), stem cell transplantation from an HLA matched sibling is the preferred treatment.
While preparing for a stem cell transplant, or if immunosuppressive treatment is to be given, it is important to monitor blood counts, since spontaneous recovery may occasionally occur, even in severely affected patients. HLA typing of the patient’s family needs to be done urgently as soon as the diagnosis is established for any patient who is a potential stem cell graft candidate.
If no HLA matched donor is available, or if the patient is over 40, immunosuppression using ATG and cyclosporin is the standard treatment.
There is an association between AA and paroxysmal nocturnal haemoglobinuria (PNH) and PNH should be tested for in all patients who present with acquired AA. In patients who have responded to immunosuppressive treatment, there is an increased risk of PNH, myelodysplasia, and AML.
Note: For a general review of Aplastic Anaemia, see How I Treat Acquired Aplastic Anemia (2012).
For the British Society for Haematology treatment guidelines for Aplastic Anaemia, see Guidelines for the diagnosis and management of aplastic anaemia. Nov 2015.
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Note: Only the electronic version is controlled. Once printed, this is no longer a controlled document.
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Topic Code: 4919
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