近30年中国文献报道恶性淋巴瘤合并自身免疫性血细胞减少症的系统性分析

Systematic analysis of cases of lymphoma with autoimmune hemocytopenia reported in the Chinese literature in the past 30 years

  • 摘要: 本文通过收集1989年1月至2019年12月国内学者发表在国内外期刊上的文献及参考文献,共纳入符合标准的140例恶性淋巴瘤(malignant lymphoma,ML)合并自身免疫性血细胞减少(autoimmune cytopenia,AIC)病例,包括溶血性贫血(autoimmune hemolytic anemia,AIHA)、免疫性血小板减少症(immune thrombocytopenia,ITP)、纯红细胞再生障碍性贫血(pure red cell aplasia,PRCA)及Evans综合征等。虽然各亚型淋巴瘤病例数均较少(n=1~28),但通过系统性分析仍得到部分有意义的发现。除免疫性中性粒细胞减少(autoimmune neutropenia,AIN)外,各类AIC均有报道,发生率依次为AIHA > ITP >冷凝集素综合征(cold agglutinin syndrome,CAS)> PRCA > Evans综合征。AIC见于各亚型淋巴瘤,但各类AIC在不同亚型发生率存在差异。CAS仅见于淋巴瘤B细胞非霍奇金淋巴瘤(B cell non Hodgkin's lymphoma,B-NHL)、AIHA、ITP,Evans综合征多见于B-NHL,PRCA则多见于T细胞非霍奇金淋巴瘤(T cell non Hodgkin's lymphoma,T-NHL)。相较于糖皮质激素等常规治疗,合并ML的AIC抗肿瘤治疗对有效率更高。恶性淋巴瘤合并AIC临床情况复杂,需引起临床关注。

     

    Abstract: The authors analyzed 140 cases of malignant lymphoma (ML) associated with autoimmune cytopenia (AIC), including autoimmune hemolytic anemia (AIHA), immune thrombocytopenia (ITP), pure red cell aplasia (PRCA), and Evans syndrome. The analysis was based on cases reported in the literature as well as the reference lists of papers in this field from 1989 to 2019 by Chinese scholars. The number of cases involving various non-Hodgkin's lymphoma subtypes was small (n=1-28). Nevertheless, interesting and sometimes unexpected differences were noted among different types of ML and AIC. All types of AIC except AIN were seen to coexist with ML, and the increasing order of incidence was as follows:warm antibody-AIHA > ITP > cold antibody-AIHA > PRCA > Evans syndrome. CAS was only seen with B-cell lymphomas, WA-AIHA, and ITP, Evans syndrome was more frequent in B-cell lymphomas and PRCA predominantly occurred with T-cell lymphomas. Anti-lymphoma treatment seemed to be more effective against AIC than conventional therapy with steroids or immunoglobulin. Although based on a literature survey, this compilation of data indicates a complex relation between lymphoma and AIC and warrants more attention and specific studies.

     

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