Richter Susann, Makovitzky Josef
Institute of Pathology, Clinic of Gynecology and Obstetrics am Klinikum der Hansestadt Rostock, University of Rostock, Südring 81, D-18059 Rostock, Germany.
Acta Histochem. 2006;108(3):221-6. doi: 10.1016/j.acthis.2006.03.016. Epub 2006 May 22.
Multiple myeloma (MM) is a neoplastic disorder characterized by proliferation of a single clone of plasma cells derived from B cells, which proliferates in the bone marrow and frequently invades the adjacent bone, producing skeletal destruction that results in bone pain and fractures. Patients with MM can furthermore present with anemia, hypercalcemia and renal failure. Non-secretory multiple myeloma (NSMM) is characterized by the absence of a monoclonal (M) protein in both the serum and urine. The reported incidence is 1-5% of all multiple myeloma cases. Development of amyloid tumors in NSMM has been described in the literature only occasionally. The clinical features of a 49-year-old female patient with NSMM and amyloid tumors in the breast, lung and rib are presented in this report. Conventional histology, Congo red staining with and without potassium permanganate pretreatment, aldehyde bisulfite-toluidine blue (ABT) reaction, sialic acid specific topo-optical reaction, toluidine blue topo-optical reaction as well as immunohistochemistry were performed. An attempt is made to explain the lack of monoclonal immunoglobulins in the serum and urine, although extensive organ amyloidosis of AL type (kappa-light chains) has been found. It is assumed that the plasmocytic plasma cells possess an excretory mechanism, which allows the pathologic immunoglobulins to be secreted either as amyloid proteins polymerizing into amyloid fibrils, or as immunoglobulin fragments that are subject to degradation as soon as they are excreted out of the tumor cell. In this paper, we review the occurrence of amyloid tumors in non-secretory multiple myeloma and, in a single case report, we confirm the existence of carbohydrate residues, including sialic acids and sulfated GAGs, in amyloid deposits.
多发性骨髓瘤（MM）是一种肿瘤性疾病，其特征为源自B细胞的单个浆细胞克隆增殖，该克隆在骨髓中增殖并经常侵犯相邻骨骼，导致骨骼破坏，进而引起骨痛和骨折。MM患者还可能出现贫血、高钙血症和肾衰竭。非分泌型多发性骨髓瘤（NSMM）的特征是血清和尿液中均不存在单克隆（M）蛋白。报道的发病率占所有多发性骨髓瘤病例的1%-5%。NSMM中淀粉样瘤的发生在文献中仅偶尔有描述。本报告介绍了一名49岁女性NSMM患者乳房、肺和肋骨出现淀粉样瘤的临床特征。进行了常规组织学检查、有无高锰酸钾预处理的刚果红染色、醛亚硫酸氢盐-甲苯胺蓝（ABT）反应、唾液酸特异性拓扑光学反应、甲苯胺蓝拓扑光学反应以及免疫组织化学检查。尽管发现了广泛的AL型（κ轻链）器官淀粉样变性，但仍试图解释血清和尿液中缺乏单克隆免疫球蛋白的原因。据推测，浆细胞具有一种排泄机制，该机制允许病理性免疫球蛋白要么作为聚合成淀粉样纤维的淀粉样蛋白分泌，要么作为免疫球蛋白片段分泌，这些片段一旦从肿瘤细胞中排出就会被降解。在本文中，我们回顾了非分泌型多发性骨髓瘤中淀粉样瘤的发生情况，并且在一个个案报告中，我们证实了淀粉样沉积物中存在包括唾液酸和硫酸化糖胺聚糖在内的碳水化合物残基。
