Bone marrow failure syndromes: paroxysmal nocturnal hemoglobinuria.
This article discusses the etiology of paroxysmal nocturnal hemoglobinuria (PNH) and its relationship to marrow hyperplasia. The author posits that the defining clinical pathology of PNH (ie, complementmediated intravascular hemolysis) is an epiphenomenon that is a consequence of an orchestrated response (ie, natural selection of PIGA-mutant stem cells) to a specific type of bone marrow injury (ie, immune mediated). Management of PNH is discussed also.
Parker
Charles J
CJ
Division of Hematology and Bone Marrow Transplantation, Department of Medicine, University of Utah School of Medicine, 50 North Medical Drive, Salt Lake City, UT 84132, USA. charles.parker@hsc.utah.edu