Bashir Bashir Abdrhman, Othman Suhair A, Malik Amel Aziz
Department of Hematology, Faculty of Medical Laboratory Sciences, Port Sudan Ahlia College, Port Sudan, Sudan.
Department of Pediatric, Faculty of Medicine, Red Sea University, Port Sudan, Sudan.
Sudan J Paediatr. 2021;21(1):89-94. doi: 10.24911/SJP.106-1583095341.
Evans syndrome is a condition in which autoimmune-mediated red blood cells and platelet destruction happens consecutively. It may be associated with a reduction in neutrophil count as a result of immune neutropenia. No sex preference is known and it presents in all ages and any ethnic cohort. Generally, this syndrome tends to be chronic and is characterised by remission and exacerbation. We document a case of the immune-mediated disease associated with Epstein-Barr virus infection in an 8-year-old boy from eastern Sudan who presented with both immune thrombocytopenia purpura and autoimmune haemolytic anaemia. Complete blood count and peripheral blood picture revealed features consistent with immune haemolytic anaemia (rouleaux formation and spherocytes) and thrombocytopenia. Direct anti-human globulin test and indirect anti-human globulin test were positive. Evans syndrome is a potentially life-threatening condition due to the concomitant existence with antiplatelet and anti-erythrocyte antibodies distinguished by a positive antiglobulin test and possibly linked to other autoimmune or lymphoproliferative diseases.
伊文氏综合征是一种自身免疫介导的红细胞和血小板相继遭到破坏的病症。由于免疫性中性粒细胞减少，它可能伴有中性粒细胞计数降低。尚无性别偏好，各年龄段和任何种族群体均可出现。一般来说，该综合征往往呈慢性，其特征为缓解期和加重期交替。我们记录了一例来自苏丹东部的8岁男孩患与爱泼斯坦-巴尔病毒感染相关的免疫介导疾病的病例，该男孩同时患有免疫性血小板减少性紫癜和自身免疫性溶血性贫血。全血细胞计数和外周血涂片显示出与免疫性溶血性贫血（缗钱状形成和球形红细胞）及血小板减少相符的特征。直接抗人球蛋白试验和间接抗人球蛋白试验均呈阳性。伊文氏综合征是一种潜在的危及生命的病症，因其同时存在抗血小板和抗红细胞抗体，以抗球蛋白试验阳性为特征，并且可能与其他自身免疫性或淋巴增殖性疾病相关。
