All patients will require blood product support and aggressive management of infection. The prognosis of severe aplastic anaemia managed with supportive therapy only is poor and more than 50% of patients die, usually in the first year. The curative treatment for patients under 30 years of age with severe idiopathic aplastic anaemia is allogeneic bone marrow transplantation if there is an available donor Those with a compatible sibling donor should proceed to transplantation as soon as possible; they have a 75-90% chance of long-term cure.
In older patients, immunosuppressive therapy with ciclosporin and antithymocyte globulin gives 5-year survival rates of 75%. Such patients may relapse or other clonal disorders of haematopoiesis may evolve, such as paroxysmal nocturnal haemoglobinuria myelodysplastic syndrome and even acute myeloid leukaemia They must be followed up long-term.…
