Castleman病的临床病理观察

目的 研究Castleman病临床病理学的特点,探讨其鉴别诊断和发病机制。方法 观察26例Castleman病的病理形态,用EnVision两步法对其中20例行CD45RO、CD45RA、CD20,以及κ、λ轻链免疫组织化学染色,结合其临床表现、治疗进行分析,并获得16例的随访资料。结果 临床表现为伴有贫血、血沉快、血清球蛋白异常、肝脾肿大、皮疹以及肺和肾损害,1例因并发肺损害和肾衰竭死亡。病变呈局灶性20例、多中心性6例;透明血管型19例、浆细胞型4例和混合细胞型3例。多中心性包括了4例浆细胞型和2例混合细胞型;局灶性20例中,13例仅有单发淋巴结肿大,且均为透明血管型,预后良好。另7例伴副肿瘤性天疱疮者,有血清球蛋白异常(4／7)及肺脏异常(5／7例),透明血管型6例和混合型1例,伴套区淋巴细胞和树突状网织细胞,小血管和浆细胞增生。术后皮损好转,肺损害无改变。免疫组织化学可见κ和λ两种轻链的表达。结论 Castleman病为淋巴组织增生性疾病,临床和病理上要与多种疾病鉴别,且病理的鉴别尤其重要。它的多器官受累可能与自身免疫疾病的发病机制相似。

A clinicopathological study of Castleman's disease

OBJECTIVE: To study the pathogenesis and the differential diagnosis of Castleman's disease. METHODS: Histopathology, immunohistochemical staining and clinical courses of 26 cases of Castleman's disease (CD) were studied with follow-up study of 16 cases. RESULTS: The present study included 6 cases of multicentric type, 20 cases of localized type in the clinical aspects and 19 cases with hyaline vascular type, 4 cases with plasma cell type, 3 cases with mixed type in the histologic aspect. The Multicentric type presented systemic lymphadenopathy, anemia, hyperglobulinemia, hepatosplenomegaly, skin changes, and lung disorder and kidney disfunction, of which 1 case died of respiratory and renal insufficiency. 13 of the 20 localized cases were of the hyaline vascular type, and with good prognosis. 7 of the 20 cases showed paraneoplastic pemphigus associated with hyperglobulinemia (4/7) and lung disease (5/7). The pathologic features composed of proliferation of the mantle zone B cell, follicular dendritic cell, plasma cell and small vessels. In immunohistochemical staining, kappa and lambda light chains were detected in each CD case. CONCLUSIONS: Many diseases are similar to CD clinicopathologically. It is important to make differential diagnosis through pathological study. Castleman's disease is a lymphoproliferative disorder. The pathogenesis of this multicentric disorder may be associated with autoimmune disease.