Clinical aspects and treatment of idiopathic aplastic anemia

The clinical picture of IAA generally reflects the extent of HSC loss and the subsequent cytopenias. Typically, a patient with severe IAA presents with bruising and mucosal bleeding, anemia and septic episodes (bacterial or fungal), but without hepatosplenomegaly. The differential diagnosis of IAA, as well as hypoplastic MDS, includes inherited BMF syndromes, the aplastic form of childhood ALL, infectious and malignant processes that may infiltrate the bone marrow. Thus, the diagnosis of IAA is made eventually by exclusion. The contemporary treatment of IAA is dictated by the severity of IAA (as determined by the degree of pancytopenia, reticulocyto-penia and bone marrow cellularity) and by the patient's age. HSC transplantation (HSCT) from an HLA-identical sibling or from an alternative donor is the treatment of choice for younger patients with severe IAA, and offers better than 65%

long-term survival. In the absence of an appropriate donor, or when the patient is older or the disease milder, immu-nosuppressive treatment (in particular the combination of ALG/ATG and cyclosporin A) results in a complete or partial response in most cases. As well as PNH (see below), IAA bears a significant risk of late clonal disorders, especially after immunosuppressive therapy: the risk of MDS and acute myeloid leukemia (AML) is 7% 10 years after immunosuppression, and the risk of tumors of other organs brings the total risk to 18% (compared with 3.1% after bone marrow transplantation).

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