Course of the Disease

The onset of PNH is insidious. There is no familial tendency. Venous thrombosis is more often responsible for death than bone marrow failure in patients with PNH. Spontaneous long-term remission or leukemia transformation may occur in some patients.

Patients with classic PNH may have cytopenia of one or all blood cell lineages and the degree of bone marrow failure may vary from mild to severe. About 15% of patients with aplastic anemia develop overt PNH; however, 35-50% of aplastic anemia patients may have flow cytometric evidence of deficiency of GPI-linked molecules at some stage of their disease as evidence of subclinical PNH.

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