儿童再生障碍性贫血-阵发性睡眠性血红蛋白尿综合征临床分析

为探讨儿童再生障碍性贫血 -阵发性睡眠性血红蛋白尿 (AA PNH)综合征的临床及实验室检查特点 ,特总结近年我科收治的 5例AA PNH综合征患儿的病历资料 ,从临床、血象、骨髓象、溶血方面的检查、CD5 9+细胞等方面分析其特点。结果显示儿童AA PNH综合征以学龄儿童为多见 ,本文报导 1例发病年龄 1岁半 ,为目前文献报导中年龄最小者 ;儿童AA PNH以AA伴PNH特征多见 ;临床上有严重贫血、肝脾稍大等特点 ;实验室检查可见全血细胞减少、Ret大多不低或升高、血片中可见有核红细胞 ;骨髓红系增生 ,粒红比例倒置、巨核系统大多增生低下 ;Hams试验、蔗糖水溶血试验、尿Rous试验、蛇毒因子 (Cof)溶血试验可阳性 ;血细胞膜CD5 9-细胞增多 ,粒细胞膜CD5 9-细胞比红细胞膜出现早。因此 ,可以结论　儿童AA PNH综合征多见于学龄期 ,以AA伴PNH特征多见。临床上对于全血细胞减少、骨髓红系增生、血Ret升高、血片中可见有核红细胞的患儿 ,应进一步做与PNH有关的检查 ,并综合分析 ,CD5 9-粒细胞增多具有早期诊断价值

An analysis on clinical characteristics in children with aplstic anemia-paroxysmal nocturnal hemoglobiuria syndrome

In order to find out the clinical characteristics in children with aplastic anemiaparoxysmal nocturnal hemoglobinuria (AA PNH) syndrome, we analysis clinical and experimental data (including blood routine test, bone marrow analysis, CD 59 test in peripheral blood, etc) of 5 children with AA PNH syndrome. Results showed that a case with onset of symptoms at the age of 1 and a half years, He is the youngest patient by now. The other cases were juvenile. Most cases manifested aplastic anemia with PNH characteristics. Clinical features included serious anemia and hepatosplenomegaly. Blood routine test showed that peripheral blood cells decreased, blood rets were normal or high. There were erythrocytoblast in peripheral blood smears. There were hyperplastic erythron and hypoplastic megacaryocyte in BM. Hams tests, urine routine tests and Cof's tests were positive. CD 59 leukocytes increased earlier than erythrocytes. Therefore The clinical characteristics of AA PNH syndrome in children were aplastic anemia with PNH characteristics. The early diagnosis should be based on the increased CD 59 of peripheral leukocytes.