多中心Castleman病临床病理分析

ANALYZE THE CLINICAL MANIFESTATIONS AND PATHOLOGICAL FEATURE OF MULTICENTER CASTLEMAN DISEASE

目的:分析多中心Castleman病(MCD)的临床病理特点。方法:MCD患者5例,其中浆细胞型(PC)3例,透明血管型(HV)2例,均为男性,年龄43岁～62岁(中位年龄50岁)。4例接受COP化疗,并分别应用糖皮质激素、沙利度胺+干扰素维持,1例放弃治疗。结果:临床情况5例分别不同程度表现发热、乏力、盗汗等非特异性全身症状,5例均存在多部位浅表淋巴结肿大,3例脾脏肿大。实验室结果:出现贫血及肾功能损害各有3例,4例患者出现多克隆高γ球蛋白血症,4例患者骨髓浆细胞或嗜酸粒细胞增多。治疗转归:1例4疗程COP治疗后生存8 a,2例治疗后达PR,带病生存4个月、8个月,仍随访中。结论:多中心CD常累及多系统损伤,且表现较淋巴结肿大为早,PC型全身症状相对较重,致使MCD早期诊断存在困难,确诊须经淋巴结病理活检证实。

Objective: To analyze the clinical manifestations and pathological feature of multicenter Castleman disease (MCD). Methods: There are five cases of MCD, and three of them are plasmacyte type (PC) , two of them are hyaline vessel type (HV). Five cases are all male and the age from 43 to 62 year old. Four cases of MCD accept chemotherapy including COP, and they maintain therapy with glucocorticoid or thalidomide plus interferon. One patient give up treatment. Results: Clinical stat: five patients is characterized by different fever, faint and night sweats etc. aspecific clinical symtom. And the five cases all have lymphadenopathy, as well as three of them have be found splenomegaly. Laboratory result: there are three patients are found to be anaemia and renal function lesion. Four patients presented hypergammaglobulinemia . In the bone marrow, there four cases show plasmocyte increasing or eosinophilia. Therapeutic efficacy: one case free survive eight years after COP four courses; two patients achieved partly remission after chemotherapy and still be following-up. Conclusion: MCD is usually characterized by polysystem damage in clinical manifestations with enlarged lymph nodes. If systemic manifestations occur earlier than lymphadenopathy, it is difficulty to diagnose MCD. This disorder must be proved by lymph node biopsied.