论文部分内容阅读
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 clinicopathological features of multicenter Castleman disease (MCD). Methods: There were 5 patients with MCD, including 3 cases of plasma cell type (PC) and 2 cases of clear blood vessel type (HV). All of them were male, ranging in age from 43 to 62 years (median 50 years). Four patients received COP chemotherapy and were treated with glucocorticoid, thalidomide and interferon respectively, and one patient gave up treatment. Results: There were 5 cases of nonspecific systemic symptoms of fever, fatigue, night sweats, etc. in 5 cases. There were multiple superficial lymph nodes in 5 cases and swollen spleens in 3 cases. Laboratory Results: There were 3 cases of anemia and renal dysfunction, 4 cases of polyclonal hypergammaglobulinemia, 4 cases of bone marrow plasma cells or eosinophilia. Treatment outcome: 1 case of 4-course COP treatment survival 8 a, 2 cases of PR after treatment, survival of the disease for 4 months, 8 months, still follow-up. CONCLUSIONS: Multi-center CD often involves multiple system injuries and manifests itself earlier than lymphadenopathy. PC-like systemic symptoms are relatively severe, making early diagnosis of MCD difficult. Diagnosis must be confirmed by biopsy of lymph nodes.