Takahashi K, Naito M, Takatsuki K, Kono F, Chitose M, Ooshima S, Mori N, Sakuma H, Uchino F
Acta Pathol Jpn. 1987 Jan;37(1):141-54. doi: 10.1111/j.1440-1827.1987.tb03142.x.
An autopsy case of multiple myeloma, IgA kappa type, accompanying systemic crystal-storing histiocytosis and generalized amyloidosis, is reported. Besides multiple destructive lesions in the skeletal bones, nodular myeloma cell infiltrates were scattered in the liver, spleen, and both kidneys. Not only in these lesions but also in the reticuloendothelial organs, crystal-storing macrophages appeared dispersively or in clusters. Electron microscopically, numerous crystalline inclusions contained in the cytoplasm of macrophages were membrane-bound and of variable configuration, comprising of a homogeneous electron-lucid material. Enzyme cytochemically, almost all of the inclusions showed acid phosphatase activity. On the basis of the results obtained from the immunohistochemical, immunofluorescent and immunoelectron microscopic studies, it was considered that the crystalline inclusions stored in the macrophages were derived from IgA kappa immunoglobulin secreted from the myeloma cells and were formed within secondary lysosomes by crystallization during lysosomal digestion and degradation of the ingested immunoglobulin by macrophages. Generalized amyloidosis developed in different sites from those of the crystal-storing histiocytosis and were proven immunohistochemically to belong to AL amyloidosis probably derived from a certain group of A kappa precursor protein.
报告了一例IgA κ型多发性骨髓瘤尸检病例，伴有全身性晶体贮积性组织细胞增多症和全身性淀粉样变性。除了骨骼中的多发性破坏性病变外，结节性骨髓瘤细胞浸润散在于肝脏、脾脏和双肾中。不仅在这些病变中，而且在网状内皮器官中，晶体贮积巨噬细胞也呈散在或成簇出现。电子显微镜下，巨噬细胞胞质内的大量晶体包涵体被膜包绕，形态各异，由均质的电子透明物质组成。酶细胞化学研究显示，几乎所有的包涵体都具有酸性磷酸酶活性。根据免疫组织化学、免疫荧光和免疫电子显微镜研究结果，认为巨噬细胞内贮存的晶体包涵体来源于骨髓瘤细胞分泌的IgA κ免疫球蛋白，是巨噬细胞摄取免疫球蛋白后在溶酶体消化降解过程中通过结晶在次级溶酶体内形成的。全身性淀粉样变性发生在与晶体贮积性组织细胞增多症不同的部位，免疫组织化学证实属于AL淀粉样变性，可能来源于某一组A κ前体蛋白。
