- 期刊
Raroxysmal Nocturnal Hemoglobinuria: Report Of One Case
發作性夜間血紅素尿症：一病報告
摘要
夜間發作性血紅素尿症為一種肇因於骨髓幹細胞缺陷之慢性溶血性貧血，其很少發生於小孩。本文報告一例小兒病例，其為六歲女孩，主訴臉色蒼白及全身瘀斑，經過末梢血液及骨髓檢查後，於再生不良性貧血之臆斷下接受支持性療法。隨後因兩次溶血性輸血反應，以及 Ham Test 及 Sugar Water Test 皆為陽性，因此確立診斷。本文就此疾病之病因、臨床表徵、診斷及治療參考文獻作一簡單的回顧。
關鍵字
無資料並列摘要
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, pat ho genesis, diagnosis and management of PNH is reviewed briefly.
延伸閱讀
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