Abstract
Most cases of aplastic anaemia in children are idiopathic. Although individually rare, the inherited bone marrow failure syndromes (IBMFSs) collectively constitute an important minority of presenta-tions with bone marrow failure. This chapter describes the epidemiolo-gy, clinical presentations, investigation, diagnosis and management of aplastic anaemia and IBMFSs in children. Supportive care and defini-tive management with intensive immunosuppressive treatment or hae-mopoietic stem cell transplantation, complications and prognosis are described. The presentations and management of the least rare IBMFSs, Fanconi anaemia and dyskeratosis congenita, are described and other even rarer conditions are mentioned briefly.
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