Castleman病31例临床病理分析

Clinicopathologic features of Castleman disease: an analysis of 31 cases

目的探讨Castleman病(CD)临床病理特征、诊断与鉴别诊断及病因。方法对31例CD进行临床特征、病理形态学特点、免疫组化、EB病毒原位杂交检测及分析。结果 31例CD病例中,22例为单中心型,9例为多中心型;透明血管型25例,浆细胞型3例,透明血管浆细胞混合型3例。本组病例中,发生于腹膜后者11例,该部位是CD的好发部位之一(35.5%)。31例中27例有完整随访资料,其中18例单中心型术后均未复发,9例多中心型中1例复发、1例死于心脏疾病。结论 CD较少见,明确诊断需依靠病理学检查,准确的临床病理分型,对患者的治疗和预后具有重要意义。单中心型CD治疗以局部切除为主,预后良好,多中心型CD治疗以联合化疗或免疫治疗为主,预后较差,美罗华在多中心型CD治疗方面疗效显著。

Objective To discuss the clinicopathologic features, diagnosis, differential diagnosis and etiology of Castleman disease. Methods A retrospective analysis of the clinical features, pathological morphology,immunohistochemistry and Epstein-Barr virus was carried out in 31 cases of Castleman disease. Results There were 22 cases of unicenrtic Castleman disease and 9 cases of multicentric Castleman disease. Pathologically,there were 25 cases of hayline vascular types,3 cases cyic of plasmat type and 3 cases of mixed type in all Castleman disease. In this series,there were 11 cases which occurred in retroperitoneum, which was the main site of Castleman disease（ 35. 5%）.Immunohistochemistry can assist diagnosis and differential diagnosis. The results of EBER-ISH showed negative signal in 15 cases of Castleman disease. There were 27 cases with complete follow-up data,including 18 cases of unicenrtic type which were healed by surgical resection without recurrence; in all cases of multicentric Castleman disease; 1 case recurred,other multicentric cases achieved sustained remission; 1 case died of heart disease. Conclusions Castleman disease is a rare lymphoproliferative lesion,and identified diagnosis depends on pathological examination. The accuracy of clinical subtype and histopathogenic type is important for treatment and outcome in the patients. Complete surgical resection is curative for unicenrtic Castleman disease,leading to excellent long-term outcomes. Combination chemotherapy and immunological therapy are usually used in multicentric Castleman disease,and the prognosis of these patients is poor. The use of rituximab is effective on multicentric Castleman disease.