特发性多中心型Castleman病合并自身免疫性溶血性贫血的临床特征分析OA北大核心

Objective To investigate the clinical characteristics,treatment responses,and prognosis of patients with idiopathic multicentric Castleman disease(iMCD)complicated by autoimmune hemolytic anemia(AIHA).Methods The patients diagnosed with iMCD in Peking Union Medical College Hospital from January 2010 to December 2023 and having complete baseline blood routine data were retrospectively enrolled in the study.The iMCD patients were further assigned into AIHA and non-AIHA groups based on baseline laboratory examina-tions,and the clinical characteristics and prognosis were compared between the two groups.Results A total of 341 patients with iMCD were enrolled in this study,including 277(81.2%)exhibiting anemia at baseline.Five(1.8%)patients were identified as having iMCD-AIHA,all of whom were iMCD-not otherwise specified type complicated by warm antibody-type AIHA,and two of them were simultaneously diagnosed with Evans syn-drome.The timing relationship between the first onset of AIHA and iMCD diagnosis varied,with 2 patients experiencing their first hemolytic episode prior to the diagnosis of iMCD.In terms of treatment,the therapy targe-ting iMCD was effective in alleviating AIHA.The AIHA group had a poorer prognosis(HR=4.61,95%CI=1.08-19.80,P=0.040)and a lower 5-year survival rate(90%vs.60%,P=0.024)than the non-AIHA group.Conclusions iMCD-AIHA is clinically rare,and AIHA can occur at different stages of iMCD.Although the primary treatment principle remains targeting iMCD,great attention should be paid to the exacerbation of immune dysfunction caused by AIHA and the possible adverse prognosis it may bring.