Min Congcong, Liu Ailing, Xu Yushuang, Yu Yanan, Xu Yonghong, Mao Tao, Ding Xueli
Department of Gastroenterology, the Affiliated Hospital of Qingdao University, Qingdao, China.
Front Med (Lausanne). 2025 Jun 16;12:1537944. doi: 10.3389/fmed.2025.1537944. eCollection 2025.
POEMS syndrome is a rare hematologic disorder related to plasma cell dyscrasia. The Castleman disease variant of POEMS syndrome is extremely rare and often misdiagnosed. In this study, we aim to present a noteworthy case of POEMS syndrome mainly manifested as multiple pleural effusion and renal impairment without M protein.
A 47-year-old woman was admitted to the hospital with a 7-month history of lower extremity edema and 3 months of abdominal distension. Computed tomography revealed poly-serosal effusion and hepatosplenomegaly, while ultrasound showed multiple superficial lymphadenopathies. Serum protein electrophoresis and bone biopsy indicated no evidence of monoclonal plasma cell proliferation. Pathological results obtained from lymph node biopsy revealed Castleman disease (CD). The patient was ultimately diagnosed with the Castleman disease variant of POEMS syndrome without M protein. Renal function gradually declined in the later stages of the disease. After transferring to another hospital, the patient received a VPD chemotherapy regimen (Pomalidomide, Bortezomib, and Dexamethasone) and hemodialysis. Effusions in multiple serosal cavities were reduced, and renal function improved significantly following active treatment.
POEMS syndrome without M protein is often misdiagnosed as other conditions. In patients presenting with multiple systemic manifestations, the possibility of POEMS syndrome or CD should be considered.
POEMS综合征是一种与浆细胞异常增生相关的罕见血液系统疾病。POEMS综合征的Castleman病变异型极为罕见，且常被误诊。在本研究中，我们旨在呈现一例以多发性胸腔积液和肾功能损害为主要表现且无M蛋白的POEMS综合征的显著病例。
一名47岁女性因下肢水肿7个月、腹胀3个月入院。计算机断层扫描显示多浆膜腔积液和肝脾肿大，而超声显示多处浅表淋巴结病。血清蛋白电泳和骨活检未发现单克隆浆细胞增殖的证据。淋巴结活检的病理结果显示为Castleman病（CD）。该患者最终被诊断为无M蛋白的POEMS综合征的Castleman病变异型。疾病后期肾功能逐渐下降。转至另一家医院后，患者接受了VPD化疗方案（泊马度胺、硼替佐米和地塞米松）及血液透析。积极治疗后，多浆膜腔积液减少，肾功能显著改善。
无M蛋白的POEMS综合征常被误诊为其他疾病。对于出现多种全身表现的患者，应考虑POEMS综合征或CD的可能性。
