Striking effectiveness of siltuximab-based treatment in refractory idiopathic multicentric Castleman disease resembling IgG4-related disease.

IF 1.8 4区医学 Q3 INFECTIOUS DISEASES

Journal of Chemotherapy Pub Date : 2025-08-05 DOI:10.1080/1120009X.2025.2542615

Xing Gong, Weiwei Yan, Feng Zhang, Dongdong Zhang

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引用次数: 0

Abstract

Castleman disease (CD) and immunoglobulin G4-related disease (IgG4-RD) are rare systemic immune-mediated disorders that share similar clinical manifestations and overlapping pathological features. We present a case of insidious idiopathic multicentric CD (iMCD) with elevated serum IgG4 levels, characterized by a persistent dry cough and widespread lymphadenopathy. A 38-year-old male patient exhibited persistent dry cough, generalized lymphadenopathy, and fatigue. An inguinal lymph node biopsy showed plasma cell-type MCD characteristics, such as atrophic lymphoid follicles and significant plasma cell infiltration. Immunohistochemical staining revealed positivity for IgG, CD38, and CD138, with over 50 IgG4-positive plasma cells per high-power field and an IgG4/IgG ratio of 40%, confirming the diagnosis of the idiopathic plasmacytic lymphadenopathy (IPL) subtype of multicentric Castleman disease (iMCD-IPL). After receiving a siltuximab-based regimen followed by radiotherapy, the patient achieved a notable partial remission. We present a case illustrating the significant efficacy of siltuximab-based therapy in iMCD-IPL, resembling IgG4-related disease.

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西妥昔单抗治疗难治性特发性多中心Castleman病（类似igg4相关疾病）的惊人疗效

Castleman病（CD）和免疫球蛋白g4相关疾病（IgG4-RD）是罕见的系统性免疫介导疾病，具有相似的临床表现和重叠的病理特征。我们报告一例潜伏的特发性多中心性CD (iMCD)，血清IgG4水平升高，以持续干咳和广泛的淋巴结病为特征。38岁男性患者表现为持续干咳，全身淋巴结肿大，疲劳。腹股沟淋巴结活检显示浆细胞型MCD特征，如萎缩的淋巴滤泡和明显的浆细胞浸润。免疫组化染色显示IgG、CD38和CD138阳性，高倍视场IgG4阳性浆细胞超过50个，IgG4/IgG比值为40%，证实多中心Castleman病（iMCD-IPL）特发性浆细胞性淋巴结病（IPL）亚型的诊断。在接受以西妥昔单抗为基础的放疗方案后，患者获得了显著的部分缓解。我们提出一个病例，说明基于西妥昔单抗的治疗iMCD-IPL的显着疗效，类似于igg4相关疾病。

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来源期刊

Journal of Chemotherapy 医学-药学

CiteScore

3.70

自引率

0.00%

发文量

144

审稿时长

6-12 weeks

期刊介绍： The Journal of Chemotherapy is an international multidisciplinary journal committed to the rapid publication of high quality, peer-reviewed, original research on all aspects of antimicrobial and antitumor chemotherapy. The Journal publishes original experimental and clinical research articles, state-of-the-art reviews, brief communications and letters on all aspects of chemotherapy, providing coverage of the pathogenesis, diagnosis, treatment, and control of infection, as well as the use of anticancer and immunomodulating drugs. Specific areas of focus include, but are not limited to: · Antibacterial, antiviral, antifungal, antiparasitic, and antiprotozoal agents; · Anticancer classical and targeted chemotherapeutic agents, biological agents, hormonal drugs, immunomodulatory drugs, cell therapy and gene therapy; · Pharmacokinetic and pharmacodynamic properties of antimicrobial and anticancer agents; · The efficacy, safety and toxicology profiles of antimicrobial and anticancer drugs; · Drug interactions in single or combined applications; · Drug resistance to antimicrobial and anticancer drugs; · Research and development of novel antimicrobial and anticancer drugs, including preclinical, translational and clinical research; · Biomarkers of sensitivity and/or resistance for antimicrobial and anticancer drugs; · Pharmacogenetics and pharmacogenomics; · Precision medicine in infectious disease therapy and in cancer therapy; · Pharmacoeconomics of antimicrobial and anticancer therapies and the implications to patients, health services, and the pharmaceutical industry.