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儿童Castleman病4例临床诊疗分析
Clinical diagnosis and treatment of four cases of Castleman disease in children
分析2021年3月—2024年12月广州医科大学附属妇女儿童医疗中心收治的4例Castleman病患儿的临床诊疗经过。其中单中心型3例,分别表现为腹痛伴回盲部溃疡、合并经典型霍奇金淋巴瘤及胆总管囊肿。重型特发性多中心型1例,颈部和腋窝区淋巴结受累,表现为发热、腹胀、全身浮肿,并伴有乏力、贫血、肝功能不全、多浆膜腔积液等全身表现。3例单中心型均手术完整切除肿大淋巴结,腹痛病例后续予肠内营养、沙利度胺口服治疗,合并霍奇金淋巴瘤者按霍奇金淋巴瘤方案化疗。重型特发性多中心型病例采用利妥昔单抗联合长春新碱、吡柔比星、环磷酰胺、泼尼松治疗。随访时间为6~30个月,4例均完全缓解。儿童Castleman病临床症状缺乏特异性,单中心型主要采用手术切除治疗,多中心型治疗方案多样,整体预后良好,Castleman病合并消化道溃疡与重型特发性多中心型病例的最佳治疗方案仍需进一步探索。
The clinical diagnosis and treatment processes of four children with Castleman disease admitted to Guangzhou Women and Children's Medical Center, Guangzhou Medical University, from March 2021 to December 2024 were retrospectively analyzed. Among them, three cases were unicentric Castleman disease, presenting as abdominal pain with ileocecal ulceration, concomitant classical Hodgkin lymphoma, and choledochal cyst, respectively. One case was severe idiopathic multicentric Castleman disease involving lymph nodes in the neck and axillary regions, presenting with fever, abdominal distension, generalized edema, fatigue, anemia, hepatic dysfunction, polyserous effusion, and other systemic manifestations. The three unicentric cases underwent complete surgical resection of enlarged lymph nodes. The case with abdominal pain received subsequent enteral nutrition and oral thalidomide therapy. The case complicated with Hodgkin lymphoma received chemotherapy according to the Hodgkin lymphoma protocol. The severe multicentric case was treated with rituximab combined with vincristine, pirarubicin, cyclophosphamide, and prednisone. All four cases achieved complete remission during 6 to 30 months of follow-up. Clinical manifestations of Castleman disease in children lack specificity. Surgical resection remains the main treatment for unicentric cases, while treatment regimens for multicentric cases are more varied. The overall prognosis is favorable. Further studies are needed to determine the optimal treatment for Castleman disease complicated by gastrointestinal ulcers and severe idiopathic multicentric Castleman disease.
Castleman病 / 消化道溃疡 / 治疗 / 儿童
Castleman disease / Gastrointestinal ulcer / Treatment / Child
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