Paroxysmal nocturnal hemoglobinuria: report of one case
Lu, J.C.; Lin, M.T.
Zhonghua Minguo Xiao Er Ke Yi Xue Hui Za Zhi Zhonghua Minguo Xiao Er Ke Yi Xue Hui 30(5): 323-325
1989
ISSN/ISBN: 0001-6578 PMID: 2637613 Document Number: 3511
Paroxysmal nocturnal hemoglobinuria (PNH) is a complex stem cell disorder and its occurrence in childhood is quite uncommon. A 6-year-old girl with pancytopenia was presented. There is no nocturnal hemoglobinuria or other symptoms of chronic hemolysis. Bone marrow examination revealed mild hypocellularity initially, and a tentative diagnosis of aplastic anemia was made. This patient received conventional therapy with uneventful course. Two consecutive episodes of hemolytic transfusion reaction were noted and positive sugar water test and Ham,s test lead the clue of PNH. The literature on the clinical manifestation, pathogenesis, diagnosis and management of PNH is reviewed briefly.
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