Wang P, Hornstein O P, Schricker K
Hautarzt. 1976 Sep;27(9):441-8.
A dermal leiomyosarcoma associated with a still asymptomatic osteomedullary plasmocytoma with IgA paraproteinemia develop-d rapidly in a 78-year-old man. The same paraprotein type IgA kappa was identified, with distinct decrease in concentration, in serum, sarcoma-tissue, and in the tumour-surrounding skin area by m-ans of immunoelectrophoresis, radial immunodiffusion, and Ouchterlony test. The immunochemical identity of the paraprotein, the course of the disease as followed clinically and immunochemically, and several histological criteria are in favour of monoclonal origin of the paraprotein from malignant plasmocytoma cells. The accumulation of paraprotein in the sarcoma tissue is primarily explained by the marked blood congestion of the tumour. To our knowledge no report exists in the medical literature of an association of dermal leiomyosarcoma and early paraproteinemic plasmocytoma. This coincidence may be only of chance but we rather suggest an immunopathological relation of both tumors due to a partial immune insufficiency caused by the preceding plasmocytoma, resulting in a diminished immunological "surveillance" of the organism and favouring the development of a new malignant cell population (i.e. leiomyosarcoma).
一名78岁男性患有与仍无症状的骨髓浆细胞瘤伴IgA副蛋白血症相关的皮肤平滑肌肉瘤，病情发展迅速。通过免疫电泳、放射免疫扩散和双向免疫扩散试验，在血清、肉瘤组织和肿瘤周围皮肤区域中鉴定出相同类型的IgA κ副蛋白，其浓度明显降低。副蛋白的免疫化学一致性、临床和免疫化学观察到的疾病进程以及一些组织学标准均支持副蛋白由恶性浆细胞瘤细胞单克隆起源。肉瘤组织中副蛋白的积累主要是由于肿瘤明显的血液充血。据我们所知，医学文献中尚无皮肤平滑肌肉瘤与早期副蛋白血症性浆细胞瘤关联的报道。这种巧合可能只是偶然，但我们更倾向于认为这两种肿瘤存在免疫病理关系，原因是先前的浆细胞瘤导致部分免疫功能不全，从而使机体的免疫“监视”功能减弱，有利于新的恶性细胞群体（即平滑肌肉瘤）的发展。
