![]() ![]() To obtain a broad overview of the natural history, approaches to management, and outcome, the International PNH Registry was recently established.Ĭlassic PNH. Pregnancy in a patient with PNH is complicated and requires careful management including prophylactic anticoagulation. ![]() Indefinite anticoagulation is recommended following a thromboembolic event and thrombolytic therapy should be considered for acute hepatic vein thrombosis (Budd-Chiari syndrome). Involvement of unusual sites (hepatic, mesenteric, cerebral, dermal veins) is characteristic of the thrombophilia of PNH. PNH clone size and ethnic/geographic factors appear to influence thrombophilic propensity, but a consensus on prophylactic anticoagulation has not been reached. Stem cell transplantation is potentially curative, but the decision on use is best made on a case-by-case basis because of the heterogeneous natural history of the disease. Complement inhibition by eculizumab is a promising new approach to treating the hemolytic anemia. For optimum management, the contribution of both hemolysis and marrow failure to the complex anemia of PNH should be determined. However, PNH is not a simple binary diagnosis and both flow cytometric characterization of glycosyl phosphatidylinositol–anchored protein expression on peripheral blood cells and marrow analysis are required for comprehensive disease classification. The primary clinical manifestations of paroxysmal nocturnal hemoglobinuria (PNH) are hemolytic anemia, marrow failure, and thrombophilia. Whalen VA Medical Center, Salt Lake City, UT the Department of Medicine, Division of Hematology, Showa University Fujigaoka Hospital, Kanagawa, Japan the Research Committee for the Idiopathic Hematopoietic Disorders, Ministry of Health, Labor, and Welfare, Government of Japan HMDS, Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom Duke University Medical Center, Department of Medicine, Division of Cellular Therapy, and the Duke University School of Medicine, Durham, NC the Division of Hematology, Washington University School of Medicine, St Louis, MO the Division of Hematology, St Jude Children's Research Hospital, Memphis, TN 1st Nazionale per la Ricerca sul Cancro, Genova, Italy the Hematology Branch, National Institutes of Health, Bethesda, MD the Research Institute for Microbial Diseases, Osaka University, Osaka, Japan and Service d'Hématologie Greffe de Moelle, Hospital Saint Louis, Paris, France. ![]() From the Division of Hematology, University of Utah School of Medicine and the George E. ![]()
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