Shaikh Hira, Mewawalla Prerna
University of Cincinnati Medical Center
Allegheny Health Network Cancer Inst
Evans syndrome is an autoimmune condition that presents with two or more cytopenias, which commonly includes autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP), with or without immune neutropenia (only in 15% of cases, according to a report) . The type of AIHA that presents in Evans syndrome is warm AIHA, in which IgG antibodies react with red blood cell (RBC) surface antigens at body temperature, as opposed to cold AIHA. In ITP, the immune system is directed against GPIIb/IIIa on the platelets. Recently, a proposition has been laid out to classify the condition as primary (idiopathic) or secondary (associated with an underlying disorder) . Secondary Evans syndrome has been associated with diseases such as systemic lupus erythematosus (SLE), common variable immunodeficiency (CVID), and autoimmune lymphoproliferative syndrome (ALPS) in Non-Hodgkin lymphoma (NHL) in patients older than 50 years, chronic lymphocytic leukemia (CLL), viral infections (such as HIV, hepatitis C) and following allogeneic hematopoietic cell transplantation. Identifying Evans syndrome as secondary when associated with a disease is important because cytopenias have been observed to be more severe when with Evans syndrome in contrast to when presenting alone as AIHA or ITP. Also, the treatment options differ according to the classification.
伊文氏综合征是一种自身免疫性疾病，表现为两种或更多种血细胞减少，通常包括自身免疫性溶血性贫血（AIHA）和免疫性血小板减少症（ITP），有或没有免疫性中性粒细胞减少症（根据一份报告，仅在15%的病例中出现）。伊文氏综合征中出现的AIHA类型是温抗体型AIHA，其中IgG抗体在体温下与红细胞（RBC）表面抗原发生反应，与冷抗体型AIHA相反。在ITP中，免疫系统针对血小板上的糖蛋白IIb/IIIa。最近，有人提出将该疾病分为原发性（特发性）或继发性（与潜在疾病相关）。继发性伊文氏综合征与系统性红斑狼疮（SLE）、常见可变免疫缺陷（CVID）、自身免疫性淋巴细胞增生综合征（ALPS）等疾病有关，在50岁以上的非霍奇金淋巴瘤（NHL）、慢性淋巴细胞白血病（CLL）、病毒感染（如HIV、丙型肝炎）以及异基因造血细胞移植后也有相关报道。当与一种疾病相关时，将伊文氏综合征识别为继发性很重要，因为与单独出现AIHA或ITP相比，当伴有伊文氏综合征时血细胞减少症更为严重。此外，治疗方案根据分类而有所不同。
