20例Castleman病的临床与病理特征分析

发布时间：2019-06-20 01:25

【摘要】：目的:分析总结20例Castleman病(CD)患者的临床表现、病理特点、治疗方法及预后,以提高对CD的认识。方法:选取南京大学附属金陵医院自2010年2月至2017年4月收治的20例Castleman病患者,回顾性分析其临床特征、组织病理学改变、治疗方法及预后。结果:①20例患者均行淋巴结活检明确诊断为CD。②20例CD患者中,男11例、女9例,年龄14至73岁,中位年龄41.5岁,UCD9例,MCD11例,HV型12例,PC型8例。③9例UCD患者,透明血管型8例,浆细胞型1例,临床主要表现为局部淋巴结肿大,3例累及肺门、2例累及纵隔、4例累及腹膜后,主要有局部压迫症状如胸痛、胸闷、腹痛、腹胀等,余基本无全身症状及多系统受累表现。9例UCD患者中8例实验室检查无明显异常,仅1例合并有自身免疫性溶血性贫血。④11例MCD患者中浆细胞型7例,透明血管型4例,临床表现为全身淋巴结肿大,多数累及颈部、腋窝、纵膈、腹股沟、肠系膜等处,9例患者多伴有全身和多系统表现,包括3例肝脾肿大,4例浆膜腔积液,5例肾受累,1例骨髓细胞学结果显示嗜酸粒细胞增多性骨髓象,1例合并过敏性肉芽肿性血管炎,1例合并POEMS综合征。11例MCD患者中,7例贫血,8例CRP升高,8例低蛋白血症,5例血沉升高,2例患者检测血清IL-6均增高,1例免疫固定电泳图谱见入型单克隆IgA和游离λ链。6例行骨髓细胞学检查中,5例正常,1例嗜酸细胞增高。8例患者行抗核抗体谱和抗中性粒细胞细胞质抗体(ANCA)检测,4例阳性,其中2例表现为抗核抗体(ANA)阳性、1例为抗SSA抗体阳性、1例ANA及ANCA均阳性。⑤MCD中有5例累及肾脏,其中男4例、女1例,中位年龄42岁,均表现为蛋白尿阳性及肾功能受损。3例PC型、2例HV型。2例患者行肾活检,病理改变分别为肾小球膜增殖样病变及AA型肾淀粉样变性。⑥20例患者行CT显示病灶多呈界限清楚的圆形、类圆形软组织密度影,密度较均匀,其中3例病例可在病灶内部钙化影,3例患者行PET-CT示多发肿大淋巴结氟代脱氧葡萄糖代谢轻度增高。⑦9例UCD患者中8例行手术切除病灶,1例行放化疗综合治疗;8例痊愈,1例患者失访。11例MCD患者中,9例患者分别予糖皮质激素单药、CHOP、CTD、VRD等化疗方案治疗,2例完全缓解、2例部分缓解、4例疾病进展、1例死亡;2例行腹膜后占位切除术后分别予糖皮质激素单药和CHOP化疗,2例患者疾病进展。结论:CD确诊主要依靠病理检查,CD可发生于任何年龄段,男女发病率基本一致,且多无特异性临床表现及影像学表现,不同临床分型,临床表现有很大差异。UCD患者常无明显临床症状,多表现为单一部位淋巴结肿大,实验室检查多正常,手术切除是其主要治疗方式,且预后较好。MCD患者常表现为全身多部位淋巴结肿大,临床症状复杂,可累及多脏器、多系统,其中包括肾脏,导致不同肾脏病理类型,需要综合治疗。
[Abstract]:Objective: to analyze and summarize the clinical manifestations, pathological features, treatment and prognosis of 20 patients with (CD) with Castleman's disease in order to improve the understanding of CD. Methods: from February 2010 to April 2017, 20 patients with Castleman disease were selected from Jinling Hospital affiliated to Nanjing University. The clinical features, histopathological changes, treatment and prognosis were analyzed retrospectively. Results: all the 120 patients with CD were diagnosed by lymph node biopsy. there were 11 males and 9 females, aged 14 to 73 years, median age 41.5 years, UCD9, MCD11, HV 12, PC 8. 39 patients with UCD, 8 patients with hyaluronic vessel type, 1 patient with plasma cell type, 3 patients with local lymphadenopathy, 3 patients with hilar involvement, 2 patients with mediastinum, 4 patients with retroperitoneal involvement. There were mainly symptoms of local compression, such as chest pain, chest tightness, abdominal pain, abdominal distension, etc. 8 of 9 patients with UCD had no obvious abnormalities in laboratory examination, only 1 patient had autoimmune hemolytic anemia. In 411 patients with MCD, plasma cell type was found in 7 patients, hyaluronic vessel type in 4 patients, and the clinical manifestations were enlarged lymph nodes, mostly involving neck, axillary, mediastinum and groin. In the mesentery, 9 patients were accompanied by systemic and multisystematic manifestations, including 3 cases of hepatosplenomegaly, 4 cases of serous effusion, 5 cases of renal involvement, 1 case of bone marrow Cytology, 1 case of allergic granulomatous vasculitis, 1 case of POEMS syndrome. In 11 cases of MCD, 7 cases were anemia, 8 cases were CRP elevation, 8 cases were hypoproteemia, 5 cases were erythrocyte sedimentation rate. Serum IL-6 was increased in 2 patients, and monoclonal IgA and free 位 chain were found in 1 case of immunofixation electrophoresis. In 6 cases, 5 cases were normal and 1 case was increased in eosinophils. 8 cases were detected by antinuclear antibody spectrum and antinephrine cytoplasmic antibody (ANCA), 4 cases were positive, 2 cases were positive for antinuclear antibody (ANA) and 1 case was positive for anti-SSA antibody. Both ANA and ANCA were positive in 1 case. 5 cases of 5MCD involved the kidney, including 4 males and 1 female, with a median age of 42 years. 3 cases were PC type and 2 cases were HV type. 2 cases underwent renal biopsy. the pathological changes were bulbar membrane proliferating lesion and AA type renal amyloidosis, respectively. CT showed that most of the lesions were round and round, similar to round soft tissue density. The density was uniform, 3 cases were calcified in the lesion, 3 cases were slightly increased in fluorodeoxyglucose metabolism of multiple enlarged lymph nodes by PET-CT. Among 79 cases of UCD, 8 cases underwent surgical resection of the lesion and 1 case was treated with radiotherapy and chemotherapy. Among 11 patients with MCD, 9 patients were treated with glucocorticoid single drug, CHOP,CTD,VRD and other chemotherapy regimen, 2 cases were completely relieved, 2 cases were partially relieved, 4 cases were disease progress, 1 case died, 2 cases were treated with glucocorticoid single drug and CHOP chemotherapy respectively, and 2 cases were treated with glucocorticoid chemotherapy and CHOP chemotherapy respectively after retroperitoneal space occupying resection, 2 cases were completely relieved, 2 cases were partial remission, 4 cases were disease progress, 2 cases were treated with glucocorticoid monotherapy and CHOP chemotherapy, 2 cases were treated with glucocorticoid monotherapy and CHOP chemotherapy respectively. Conclusion: the diagnosis of CD mainly depends on pathological examination. CD can occur in any age group, and the incidence of UCD is basically the same in men and women, and there are many non-specific clinical and imaging manifestations, and there are great differences in clinical manifestations. UCD patients often have no obvious clinical symptoms, most of them are single site lymph node enlargement, most of them are normal in laboratory examination, and surgical resection is the main treatment. The prognosis of MCD patients is good. MCD patients often show multiple lymph node enlargement, complex clinical symptoms, can involve multiple organs, multiple systems, including kidney, resulting in different renal pathological types, need comprehensive treatment.
【学位授予单位】：南京大学
【学位级别】：硕士
【学位授予年份】：2018
【分类号】：R551.2

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