摘要
目的 研究再生障碍性贫血 -阵发性睡眠性血红蛋白尿综合征 (AA PNH综合征 )与典型阵发性睡眠性血红蛋白尿症 (PNH)临床特征的异同 ,加深对AA PNH综合征的认识。方法 回顾分析了 2 8例AA PNH综合征和 5 1例典型PNH的临床表现、实验室检查及治疗反应 ,并进行了对照研究。结果 AA PNH综合征与典型PNH相比 :①血栓形成、黄疸、肝脾肿大等临床表现均较轻。②网织红细胞虽较低 ,但仍高于正常 ;骨髓涂片及活检多表现为增生减低 ,但红系比例不低 ;③各溶血指标检查阳性率均较低 ,但CD5 5、CD5 9表达异常的检出率为10 0 % ,且其在红细胞、粒细胞、淋巴细胞中表达的百分率在两组患者中无明显差异。④免疫球蛋白、T细胞亚群的检测 ,两组患者均无异常。⑤两组患者对肾上腺糖皮质激素为主的治疗均反应良好。结论 AA PNH综合征虽临床表现有别于典型PNH ,但与典型PNH无本质区别 ;CD5 5、CD5 9的检测有助于提高AA
Objective To explore the differences of clinical features and relationship between aplastic anemia paroxysmal nocturnal hemoglobinuria syndrome(AA PNH syndrome)and typical paroxysmal nocturnal hemoglobinuria(t PNH).Methods A case control study on the discrepancies of clinical and laboratory features between patients with AA PNH syndrome and t PNH was carried out.Results Compared with t PNH,AA PNH syndrome showed following features:①Lower frequencies of venous thrombosis,jaundice and enlarged liver or spleen.②Higher percentages of pancytopenia and bone marrow hypoplasia.③Lower percentages of positive hemolysis tests.The percentages of CD55 and CD59 of peripheral blood cells were not significantly different in most cases of both groups.④Immunoglobulins and subgroups of T lymphocytes were normal in cases of both groups.⑤Adrenocortical hormone was effective in cases of both groups.Conclusion AA PNH syndrome shares a same pathophysiology with t PNH;CD55 and CD59 tests can improve the diagnosis of AA PNH syndrome.
出处
《中国实用内科杂志》
CAS
CSCD
北大核心
2002年第3期153-155,共3页
Chinese Journal of Practical Internal Medicine