Wille L E, Wetteland P, Förre O, Hovig T, Winnem M
Acta Med Scand. 1979;206(5):423-8. doi: 10.1111/j.0954-6820.1979.tb13539.x.
A structurally unusual, B-cell-derived malignant tumour is reported in a 24-year-old male. The gross autopsy findings were those of classical myelomatosis, with extensive and centrally located skeletal infiltrates and typical myeloma kidneys. The tumour cells were, however, morphologically very unusual. All elements were markedly pyroninophilic, indicating active protein synthesis, but typical plasma cell characteristics were lacking. The cells were ultrastructurally characterized by large and distended vacuoles, often with ribosomal granules attached, and containing an amorphous material. Virtually all tumour elements were found to be heavily loaded with IgG(kappa) in immunofluorescence studies. IgG paraprotein was also found in serum, and kappa light chains in the urine. It is concluded that the tumour represents an intermediate form between multiple myeloma and a tumour originating from the large pyroninophilic, immunoglobulin-secreting lymphocytes.
报告了一名24岁男性患有结构异常的B细胞源性恶性肿瘤。大体尸检结果符合经典骨髓瘤病,有广泛且位于中央的骨骼浸润以及典型的骨髓瘤肾。然而,肿瘤细胞在形态上非常异常。所有细胞成分均显著嗜派洛宁,表明有活跃的蛋白质合成,但缺乏典型的浆细胞特征。细胞超微结构的特点是有大的、扩张的空泡,常常附着有核糖体颗粒,并含有无定形物质。在免疫荧光研究中发现,几乎所有肿瘤成分都大量负载有IgG(κ)。血清中也发现了IgG副蛋白,尿液中有κ轻链。结论是,该肿瘤代表了多发性骨髓瘤与源自大型嗜派洛宁、分泌免疫球蛋白的淋巴细胞的肿瘤之间的一种中间形式。