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报告13例Evans 综合征,其中男3例,女10例。年龄10～68岁。病程1周至7年。其中紫癜或紫斑11例,鼻衄8例,龈血4例,黑便5例,呕血1例,黄疸4例,畏寒或发热11例,肝脾肿大4例,肝肋下1～4cm,脾肋下3～7cm。Hb<2.5g/L4例,3～6g9例。红细胞0.9～2.28×10~(12)/L。白细胞3～57×10~9/L。血小板<50×10~9/L10例,>50×10~9/L3例。网织红细胞除2例<5%,余11例均>5%。11/13例均见有核红细胞。骨髓象:增生明显活跃12例,增生极度活跃1例。粒:红比例倒置9例。多为中晚幼红细胞增生。巨核细胞成熟障碍,血小板少见。7例作Ham’s试验阴性,12例作Coombs 试验阳性。7例做免疫球蛋白测定,1例IgA、IgM 明显高于正常。3例C_3测定1例低于正常。3例淋巴细胞转化率和E-玫瑰花结均低于正常。3例CIC 测定1例明显增高。本病有贫血、出血、血小板减少,肝脾肿大,黄疸等症状,故容易与其他疾病混淆。本文部分病例曾误诊为急性
Evans syndrome was reported in 13 cases, including 3 males and 10 females. Age 10 to 68 years old. Course of 1 week to 7 years. Among them purpura or purpura in 11 cases, epistaxis in 8 cases, gingival bleeding in 4 cases, melena in 5 cases, hematemesis in 1 case, jaundice in 4 cases, chills or fever in 11 cases, hepatosplenomegaly in 4 cases, hepatic ribs 1 ~ 4cm , Spleen ribs 3 ~ 7cm. Hb <2.5g / L4 cases, 3 ~ 6g9 cases. Erythrocyte 0.9 ~ 2.28 × 10 ~ (12) / L. WBC 3 ~ 57 × 10 ~ 9 / L. Platelets <50 × 10 ~ 9 / L10 cases,> 50 × 10 ~ 9 / L3 cases. Reticulocyte addition to 2 cases <5%, more than 11 cases were> 5%. 11/13 cases were seen with erythroblasts. Bone marrow: hyperplasia was significantly active in 12 cases, hyperplasia was extremely active in 1 case. Granules: red ratio upside down in 9 cases. More for the middle and younger erythroid hyperplasia. Megakaryocyte maturation disorders, rare platelets. Ham’s test was negative in 7 cases and positive in Coombs test in 12 cases. 7 cases of immunoglobulin determination, 1 case of IgA, IgM was significantly higher than normal. One case of 3 cases C_3 was lower than normal. Three cases of lymphocyte transformation rate and E-rosette were lower than normal. 3 cases of CIC was significantly higher in 1 case. The disease has anemia, bleeding, thrombocytopenia, hepatosplenomegaly, jaundice and other symptoms, it is easy to be confused with other diseases. Some cases of this article have been misdiagnosed as acute