本文报告一例成人脾脏生发中心缺乏和大脾综合症,伴免疫功能缺陷。脾切前,临床表现为脾功能亢进,自家免疫溶血性贫血。脾切后,反复发作暴发性感染性休克。7个家庭成员中,仅一人有大脾。其他6个成员,从6岁半到77岁,虽然实验室检查均各有不同项目的异常,但均处于亚临床状态。 This article reports a case of adult spleen germinal center deficiency and splenomegaly with concomitant immune deficiencies. Spleen before the clinical manifestations of hypersplenism, their own immune hemolytic anemia. After splenectomy, repeated episodes of fulminant septic shock. Only one of the seven family members has a large spleen. The other six members, ranging from 6 years and a half to 77 years old, were all in a subclinical state although laboratory tests showed abnormalities in different items.