PNH的回顾性研究

目的 分析阵发性睡眠性血红蛋白尿症(PNH)临床表现及实验室检测各指标的诊断价值。方法 对PNH患者进行糖水试验,酸化血清溶血试验(Ham实验),尿含铁血黄素试验(Rous),血液常规,骨髓形态学检查,铁染色,网织红细胞计数测定和临床表现的分析。结果 糖水试验阳性率100％、酸化血清溶血试验(Ham实验)阳性率为100％、Rous试验阳性率为2.8％,糖水实验、Ham实验、Rous实验均反映PNH的病理特征。中、重度贫血者占83.3％。WBC减少者占44.4％,BPC减少者占50％,三系列皆减少占33.3％,BM象示增生低下者仅占5.5％,以幼红增生为主。铁染色时,铁粒幼红细胞<15％者有11例,网织红细胞计数增高。结论 用一组相关试验诊断PNH可相互印证,加上临床实践,可提高确诊率。

The Retrospective Study of Paroxysmal Nocturnal Hemoglobinuria(PNH)

Objective To analyze the significance of some tests' indices and clinical features in diagnosing paroxysmal nocturnal hemoglobinuria (PNH). Methods Ham test, sucrose hemolysis test, urine Rous test, blood routine, bone marrow picture examination,iron stain,reticulocyte count determination and clinical features were assayed in 36 patients with PNH. Results The positive rate of ham test is 100% ,the positive rate of suceose hemolysis test is 100% ,the positive rate of urine rous test is 2. 8% ,the results of all hemolysis tests can image pathological charaeteristies of PNH. the patients with arctic anemia accounted for 83. 3% ,the patients with WBC reducing accounted for 44. 4% ,the patients with BPC reducing for 50% ,the patients with three lines reducing for 33. 3%. the hypoplasia of BM,accounted for 5. 5%. when being iron stain,11 patients in the positive rate of siderocyte is blow 15%. Conclusion A group of related tests and clinical features may be helpful in the differential diagnosis of PNH.