Guo Xiaotong, Zhu Caixia, Zhang Fen, Chen Juan, Zhang Kedong
Department of Respiratory and Critical Care Medicine, The General Hospital of Ningxia Medical University, Yinchuan, China.
Department of Rheumatology, The General Hospital of Ningxia Medical University, Yinchuan, China.
Medicine (Baltimore). 2025 Apr 4;104(14):e40681. doi: 10.1097/MD.0000000000040681.
Castleman disease (CD) is a rare chronic lymphoproliferative disorder with unclear etiology and pathogenesis. It is divided into unicentric CD, which involved a single enlarged lymph node or region of lymph nodes, and multicentric CD, which involved multiple lymph node stations. Chest computed tomography (CT) scan is of great value in the diagnosis and differential diagnosis of the disease. CT scan mainly present large soft tissue mass in lungs and multiple mediastinal lymph node enlargement. Multiple ground glass nodules in both lungs are rare in CD patient.
A 48-year-old woman presented with chest tightness, shortness of breath, cough, and sputum. The chest CT scan showed multiple ground glass nodules in both lungs.
Multicentric Castleman disease was diagnosed through biopsies of the mediastinal 4R group, 7th group lymph nodes and the right inguinal lymph nodes.
Initial treatment with prednisone was administered, but due to the absence of significant radiological improvement on chest CT after 1 month, a systemic chemotherapy was initiated.
After 6 cycles of systemic chemotherapy with cyclophosphamide, vincristine, and prednisone, the patient chest CT and clinical symptoms improved. Currently, the patient is still receiving low-dose prednisone and cyclophosphamide orally for long-term maintenance treatment.
CD that presents multiple ground glass nodules in both lungs is rare. It is easily confused with other diseases, identified diagnosis is depend on pathological examination. The accuracy of clinical subtype and histopathogenic type are important for treatment and outcome.
卡斯特曼病(CD)是一种病因和发病机制不明的罕见慢性淋巴细胞增殖性疾病。它分为单中心型CD,累及单个肿大的淋巴结或淋巴结区域,以及多中心型CD,累及多个淋巴结部位。胸部计算机断层扫描(CT)对该病的诊断和鉴别诊断具有重要价值。CT扫描主要表现为肺部的大软组织肿块和多个纵隔淋巴结肿大。双肺多发磨玻璃结节在CD患者中较为罕见。
一名48岁女性出现胸闷、气短、咳嗽和咳痰症状。胸部CT扫描显示双肺多发磨玻璃结节。
通过对纵隔4R组、第7组淋巴结及右侧腹股沟淋巴结进行活检,诊断为多中心型卡斯特曼病。
最初给予泼尼松治疗,但1个月后胸部CT未见明显影像学改善,遂开始全身化疗。
采用环磷酰胺、长春新碱和泼尼松进行6个周期的全身化疗后,患者的胸部CT和临床症状有所改善。目前,患者仍在口服低剂量泼尼松和环磷酰胺进行长期维持治疗。
双肺出现多发磨玻璃结节的CD较为罕见。它容易与其他疾病混淆,明确诊断依赖于病理检查。临床亚型和组织病理学类型的准确性对治疗和预后很重要。