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本文报道了一家系三代人中6例遗传性球形红细胞增多症。临床表现均有不同程度贫血、黄疸和脾肿大。有关各项实验检查结果异常,6例实验均值如下:小球形红细胞32.3%(8～52%),Hb7.38克%(1.5～10.5克%)、红细胞直径6.3μ(6.0～7.0μ),网织红细胞7.6%(2.5～12.4%),血清胆红质2.07mg%(0.5～5.2mg%)。在一家系中发现如此多的病例尚罕见。
This article reports six cases of hereditary spherocytosis in a family of three generations. Clinical manifestations have varying degrees of anemia, jaundice and splenomegaly. The results of all the tests were abnormal. The mean values of 6 experiments were as follows: 32.3% (8-52%) of small spherical erythrocytes, 7.38% (1.5-10.5%) of Hb, 6.3μ (6.0-7.0μ) Reticulocyte 7.6% (2.5 ~ 12.4%), serum bilirubin 2.07mg% (0.5 ~ 5.2mg%). It is rare to find so many cases in one pedigree.