先天性(遗传性)球形红细胞增多症(SCTS)是红细胞(RBC)膜先天性缺陷的疾病,为先天性溶血性贫血之一,世界各地各民族中均有发现。本文综合两个单位收治之10例及所作之家系调查,报告如下。 一般资料 10例患者就诊时年龄1.5～24岁;男6例,女4例。10例均有不同程度肝脾肿大,脾脏最大肋下9cm。8例有肉眼可见之黄疸;9例有中到重度 Congenital (hereditary) polycythemia vera (SCTS) is a disease of congenital defects of the red blood cell (RBC) membrane, one of the congenital hemolytic anemias found in all ethnic groups around the world. This article combines the two units admitted to 10 cases and the pedigree investigation, the report is as follows. General information 10 patients aged 1.5 to 24 years of age; 6 males and 4 females. 10 cases have varying degrees of hepatosplenomegaly, the largest rib spleen 9cm. 8 cases of macular jaundice; 9 cases have moderate to severe