Frizzera G, Banks P M, Massarelli G, Rosai J
Am J Surg Pathol. 1983 Apr;7(3):211-31. doi: 10.1097/00000478-198304000-00001.
This report describes the nodal and extranodal lesions observed in 15 patients with a generalized disorder that had been histologically diagnosed as Castleman's disease. The disorder was characterized by severe constitutional symptoms, constant involvement of multiple peripheral lymph nodes, and frequent hepatosplenomegaly, in association with clinical and laboratory features reminiscent of a "collagen disease." The clinical course was chronic, with remissions and exacerbations in seven patients, and aggressive and fatal in eight. The material examined included multiple lymph node biopsies, four surgical specimens of spleen, one open lung biopsy, and material from four autopsies. The diagnostic morphological findings were observed in the nodes and were represented by the following histologic triad: diffuse marked plasmacytosis, from the medulla to the subcapsular areas; prominence of the germinal centers; and good preservation of the architecture. One variant of this basic pattern featured abundant immunoblasts and blood vessels. The process appears to be a systemic reactive proliferation of B-lymphocytes, perhaps resulting from faulty immune regulation. Morphologic similarities indicate a relationship between this multicentric disorder and Castleman's disease of plasmacellular type. However, there are distinct differences between them in clinical presentation and evolution, and, consequently, in therapeutic approach.
本报告描述了15例经组织学诊断为卡斯尔曼病的全身性疾病患者所观察到的淋巴结和结外病变。该疾病的特征为严重的全身症状、多个外周淋巴结持续受累、频繁出现肝脾肿大，并伴有类似“胶原病”的临床和实验室特征。临床病程呈慢性，7例患者有缓解和加重，8例患者病情进展迅速且致命。所检查的材料包括多个淋巴结活检标本、4例脾脏手术标本、1例开放性肺活检标本以及4例尸检材料。在淋巴结中观察到了诊断性形态学表现，其由以下组织学三联征构成：从髓质到被膜下区域弥漫性显著的浆细胞增多；生发中心突出；结构保存良好。这种基本模式的一种变体以丰富的免疫母细胞和血管为特征。该过程似乎是B淋巴细胞的系统性反应性增殖，可能是免疫调节异常所致。形态学上的相似性表明这种多中心性疾病与浆细胞型卡斯尔曼病之间存在关联。然而，它们在临床表现和病程发展上存在明显差异，因此在治疗方法上也有所不同。
