Abstract
This article, the fourth in a continuing education update series on hematology, reviews paroxysmal nocturnal hemoglobinuria (PNH), a clonal marrow stem cell disorder that involves erythroid, myeloid, megakaryocytic, and some lymphoid lineage. The disorder is sometimes classified as a chronic myeloproliferative syndrome because of its potential to transform into acute leukemia or one of the myelodysplastic syndromes. Clinically, the disorder manifests and is sometimes classified as a chronic hemolytic anemia. The disorder expresses itself as chronic hemolytic anemia, variable cytopenias, and venous thrombosis. Paroxysms of nocturnal hemoglobinuria occur in a minority of patients. The disorder is strongly associated with aplastic anemia, which may precede, develop concurrently with, or appear after the diagnosis of PNH.
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