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Clinical course and flow cytometric analysis of paroxysmal nocturnal hemoglobinuria in the United States and Japan.

Medicine (Baltimore)

May 2004

From the PNH Research Committee (JN, REW, CMD, SH, KMS, WFR), Duke University Medical Center, Durham, North Carolina, United States; the Research Committee for the Idiopathic Hematopoietic Disorders, Ministry of Health, Labour and Welfare, Government of Japan (JN, YK, AK, HM, MO, TK); the Department of Hematology, Tokyo Women's Medical College (HM), Tokyo; the Department of Immunoregulation, Research Institute for Microbial Diseases, Osaka University (JN, TK); the Department of Hematology and Oncology, Osaka University Graduate School of Medicine (JN, YK); the Third Department of Internal Medicine, Kinki University Medical School (AK), Osaka; the Division of Hematology, Department of Medicine, Showa University Fujigaoka Hospital (MO), Kanagawa; the First Department of Internal Medicine, Fukushima Medical University (TS), Fukushima; the Department of Hematology/Oncology, Wakayama Medical University (HNakakuma), Wakayama; the Division of Hematology, College of Medical Technology, University of Tsukuba (HNinomiya), Ibaraki, Japan.

: To determine and directly compare the clinical course of white and Asian patients with paroxysmal nocturnal hemoglobinuria (PNH), data were collected for epidemiologic analysis on 176 patients from Duke University and 209 patients from Japan. White patients were younger with significantly more classical symptoms of PNH including thrombosis, hemoglobinuria, and infection, while Asian patients were older with more marrow aplasia. The mean fraction of CD59-negative polymorphonuclear cells (PMN) at initial analysis was higher among Duke patients than Japanese patients.

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