乌干达阵发性夜间血红蛋白尿的诊断与治疗挑战
Diagnostic and Treatment Challenges of Paroxysmal Nocturnal Hemoglobinuria in Uganda.
作者信息
Lumori Boniface Amanee Elias, Muyanja Daniel
机构信息
Department of Internal Medicine, Mbarara University of Science and Technology (MUST), Mbarara, Uganda.
出版信息
Case Rep Hematol. 2019 Jan 31;2019:7897509. doi: 10.1155/2019/7897509. eCollection 2019.
INTRODUCTION
Paroxysmal nocturnal hemoglobinuria (PNH) is a very rare disorder of the hematopoietic stem cells which is often underdiagnosed. Its incidence is about 5 cases per million inhabitants in a year, and currently, there are only 1610 patients in the International PNH Registry. In this report, we describe a case of PNH in southwestern Uganda.
CASE PRESENTATION
A 34-year-old man, subsistence farmer, with a history of multiple prior presentations with anemia, jaundice, and dark-colored urine requiring blood transfusions presented to us again in July 2018 with a week's history of palpitations, dizziness, and dark-colored urine. Investigations done suggested a direct antiglobulin test- (DAT-) negative hemolytic anemia, and subsequently, flow cytometry showed a large clone of PNH. He received many blood transfusions and hematinics on several occasions during the course of his admissions.
CONCLUSIONS
Our report showed diagnostic and treatment challenges of PNH in health resource-limited setting.
引言
阵发性睡眠性血红蛋白尿（PNH）是一种非常罕见的造血干细胞疾病，常常诊断不足。其发病率约为每年每百万居民中有5例，目前国际PNH登记处仅有1610名患者。在本报告中，我们描述了乌干达西南部的一例PNH病例。
病例介绍
一名34岁的男性，以务农为生，既往有多次贫血、黄疸和深色尿病史，需要输血治疗，于2018年7月再次前来就诊，伴有一周的心悸、头晕和深色尿病史。所做检查提示直接抗人球蛋白试验（DAT）阴性的溶血性贫血，随后流式细胞术显示有大量PNH克隆。在其住院期间，他多次接受输血和补血剂治疗。
结论
我们的报告显示了在卫生资源有限的环境中PNH的诊断和治疗挑战。
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