简介：卡斯尔曼病 (CD)，称为血管滤泡性淋巴结增生，是一种罕见疾病。两种最常见的组织学亚型是透明血管亚型和浆细胞亚型。我们进行了回顾性分析来定义 CD 的临床病理特征和生存率，这是非常罕见的，重点关注我们系列的特殊性并回顾了最近的文献。方法：这是一项在突尼斯斯法克斯 Hedi Chaker 医院内科进行的 25 年来的回顾性研究。所有患者均经组织学证实该疾病。对于每个文件，我们通过填写预先设计的表格来收集一组数据。结果：纳入 18 名患者。其中男性8例，女性10例，平均年龄42.8岁。 CD 为单中心 5 例（28%），多中心 13 例（72%）。临床上，77.7%的患者存在周围淋巴结肿大，72.2%的患者存在深部淋巴结肿大。 13例患者出现全身症状，包括一般情况（4.4%）、发热（16.6%）、浆膜炎（27.7%）和皮肤受累（33.3%）。 66% 的患者临床表现伴有生物炎症综合征。血细胞计数异常12例（66%），其中贫血11例，血小板增多3例，嗜酸性粒细胞增多3例。皮肤卡波西肉瘤合并Castleman病2例，霍奇金淋巴瘤1例，血管免疫母细胞T细胞淋巴瘤1例，淋巴结T细胞淋巴瘤1例。其中3例合并结缔组织病，其中干燥综合征2例，类风湿性关节炎1例。 1例HHV8血清学阳性，呈多中心浆细胞形态。组织学上，浆细胞型占 50%，透明血管型（39%）和混合型（11%）。治疗方面，13 例患者开始大剂量皮质类固醇治疗。二线治疗中，1例因霍奇金淋巴瘤转化而采用MOPP化疗，2例多中心形式采用生物治疗（利妥昔单抗）。 2 例单中心 CD 患者接受手术切除浅表腺病。结论：Castleman 病 (CD) 是一种局部或多中心形式的非恶性淋巴增殖性疾病，具有广泛且异质的临床谱。由于缺乏临床和放射学特异性，诊断可能很困难。治疗取决于涉及手术和/或药物治疗的临床形式。© 2023 Imen Chabchoub 等人，由 Sciendo 出版。

Introduction: Castleman disease (CD), known as angiofollicular lymph node hyperplasia, is an uncommon condition. The two most common histological subtypes are hyaline vascular and plasma cell. We performed a retrospective analysis to define the clinic-pathological features and survival of CD, which is quite rare focusing on the particularities of our series with a review of the recent literature. Methods: This is a retrospective study conducted in the department of internal medicine of Hedi Chaker hospital in Sfax, Tunisia over 25 years. The disease was histologically confirmed in all patients. For each file, we collected a set of data by filling in a pre-designed form. Results: 18 patients were included. There were 8 men and 10 women with a mean age of 42.8 years. CD was monocentric in 5 cases (28%) and multicentric in 13cases (72%). Clinically, peripheral adenopathy was present in 77.7% of patients and deep adenopathy in 72.2%. Systemic signs were found in 13patients, including general condition (4.4%), fever (16.6%), serositis (27.7%), and skin involvement (33.3%). A biological inflammatory syndrome accompanied the clinical picture in 66% of patients. Abnormalities in the blood count were found in 12cases (66%), with anemia in 11cases, thrombocytosis in 3cases, and hypereosinophilia in 3cases. Cutaneous Kaposi's sarcoma was associated with Castleman's disease in 2cases, Hodgkin's lymphoma, angioimmunoblastic T-cell lymphoma, and lymph node T-cell lymphoma were found in 1case respectively. 3 of the patients had associated connective tissue diseases such as Sjögren's syndrome in 2 cases and rheumatoid arthritis in 1case. HHV8 serology was positive in 1 case with a multicentric plasma cell form. Histologically, the plasma cell form represented 50% of cases, hyaline-vascular (39% of cases), and mixed (11% of cases). Therapeutically, high-dose corticosteroid therapy was initiated in 13 cases. As a second-line treatment, MOPP chemotherapy was used in 1case due to transformation into Hodgkin's lymphoma, and biotherapy (rituximab) was used in 2cases in the multicentric form. Surgical removal of superficial adenopathy was performed in 2patients with monocentric CD. Conclusion : Castleman's disease (CD) is a non-malignant lymphoproliferation of localized or multicentric form with a wide and heterogeneous clinical spectrum. Diagnosis can be difficult due to the lack of clinical and radiological specificity. Management depends on the clinical form involving surgical and/or medical management.© 2023 Imen Chabchoub et al., published by Sciendo.