中华医学杂志（英文版）2002，Vol.115Issue(3)：398-401,4.
近十年78例新发阵发性睡眠性血红蛋白尿症的临床分析
Clinical analysis of 78 cases of paroxysmal nocturnal hemoglobinuria diagnosed in the past ten years
赵明峰 1邵宗鸿 1李克 1陈桂彬 1刘鸿 1张益枝 1和虹 1施均 1何广胜 1储榆林 1杨天楹1
作者信息
- 1. 中国医学科学院中国协和医科大学血液学研究所血液病医院,天津,300020
- 折叠
摘要
Abstract
Objective To learn more about the clinical and laboratory features of patients with paroxysmal nocturnal hemoglobinuria (PNH) diagnosed in the past ten years. Methods Clinical and laboratory data for 78 cases of PNH diagnosed from January 1990 to November 1999 in our hospital were analyzed retrospectively. Results In comparison with PNH cases reported in the 1980s, the newly diagnosed PNH cases showed the following features: (1) older age of disease onset (from 27 to 34 years); more female cases (from 18.5% to 38.5%); more cases without hemoglobinuria (from 24.2% to 38.5%). (2) No positive family hereditary history. (3) Bone marrow dysplasia, abnormal karyotype and negative sister chromatid differentiation were found in 19.2%, 12.2% and 8.9% of the PNH patients, respectively. 12.3% of the patients had bone marrow hypoplasia, and most of them had no hemoglobinuria. Ham's tests were negative in about 34.2% of the cases. CD55 and CD59 on peripheral blood cells were deficient in 100.0% of the cases, suggesting that CD55 and CD59 tests can improve the diagnosis of PNH. (4) Adrenocortical hormone was effective in 83.8% of the patients, 54.2% of whom relapsed within one year. Eight refractory and relapsed patients were treated with low dose chemotherapy (MP therapy: Melphalan 2-6?mg*d-1; Prednisone 0.5?mg*kg-1*d-1). Five (62.5%) of them showed positive responses. Bone marrow failure and other side effects were not serious in this group of patients. Conclusions PNH, an acquired blood disease seen more often among adult males, can be diagnosed more sensitively by hemocyte member CD55 and CD59 tests and treated more effectively with adrenocortical hormone or low dose chemotherapy.关键词
阵发性睡眠性血红蛋白尿症/临床特征/诊断/治疗Key words
paroxysmal nocturnal hemoglobinuria/clinical features/diagnosis/treatment分类
医药卫生引用本文复制引用
赵明峰,邵宗鸿,李克,陈桂彬,刘鸿,张益枝,和虹,施均,何广胜,储榆林,杨天楹..近十年78例新发阵发性睡眠性血红蛋白尿症的临床分析[J].中华医学杂志（英文版）,2002,115(3):398-401,4.
