特發(fā)性多中心型Castleman病合并自身免疫性溶血性貧血的臨床特征分析

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中圖分類號：R552 文獻(xiàn)標(biāo)識碼：A 文章編號：1000-503X（2025）01-0010-06

DOI：10.3881/j. issn. 1000-503X.16135

Clinical Characteristics of Patients With Idiopathic Multicentric Castleman Disease Complicated by Autoimmune Hemolytic Anemia

GAO Yuhan，LI Siyuan，DANG Yue，LI Jian，ZHANG Lu

Department of Hematology，PUMC Hospital，CAMSand PUMC，Beijing1OO730，China

Corresponding author：ZHANG Lu Tel：010-69155549，E-mail：pumczhanglu@126.com

ABSTRACT： ObjectiveTo investigate the clinical characteristics， treatment responses，and prognosis of patients with idiopathic multicentric Castleman disease（iMCD）complicated by autoimmune hemolytic anemia （AIHA）.MethodsThe patients diagnosed with iMCD in Peking Union Medical Colege Hospital from January 2010 to December 2O23and having complete baseline blood routine data were retrospectively enrolled in the study. The iMCD patients were further asigned into AIHAand non-AIHA groups based on baseline laboratory examinations，and the clinical characteristics and prognosis were compared between the two groups.ResultsA 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 syndrome.The timing relationship betweenthe first onset ofAIHAandiMCDdiagnosis varied，with2 patients experiencing their firsthemolytic episode prior to the diagnosisof iMCD.In terms of treatment，the therapy targeting iMCD was effective in alleviating AIHA. The AIHA group had a poorer prognosis （ HR=4.61 ， （204號 1.08-19.80 ， P=0.040 ）and a lower 5-year survival rate（ 90% US. 60% ， P=0.024 ）than the non-AIHA group. ConclusionsiMCD-AIHA is clinically rare，and AIHA can occur at diferent 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 possble adverse prognosis it may bring.

Keywords：idiopathic multicentric Castleman disease；autoimmune hemolyticanemia；clinical characteristics；prognosis ActaAcadMedSin，2025，47（1） ：10-15

特發(fā)性多中心型Castleman?。╥diopathicmulticen-tricCastlemandisease，iMCD）是一類罕見且高度異質(zhì)性的淋巴增殖性疾病，其主要臨床特征包括全身性炎癥狀態(tài)、多區(qū)域淋巴結(jié)病變、多克隆淋巴細(xì)胞增殖以及器官功能障礙[1]。（剩余12434字）