Wirt D P, Grogan T M, Payne C M, Kummet T D, Durie B G, Finley P R, Rollins D
Am J Clin Pathol. 1983 Jul;80(1):75-84. doi: 10.1093/ajcp/80.1.75.
A case of phagocytic, lambda light chain, plasma cell myeloma was characterized by its clinical, morphologic, cytochemical, immunologic, and cell kinetic features. A 40-year-old man presented with Coombs-negative hemolytic anemia, hepatosplenomegaly, lytic bone lesions, lambda light chain monoclonal gammopathy, and infiltration of the bone marrow by dysplastic plasma cells, 10% of which demonstrated phagocytosis of erythroid cells. Electron microscopy demonstrated myeloma cells with prominent cytoplasmic microfilaments and erythroid cells in intracytoplasmic vacuoles. The myeloma cells did not phagocytose staphylococci in vitro. Phagocytic and nonphagocytic myeloma cells were tartrate-sensitive, acid-phosphatase positive, alpha-napthyl butyrate esterase negative, and did not form E rosettes or EAox(IgG) rosettes. The tumor cells were Tdt, Ia antigen, and SIg negative. Immunofluorescent staining for cytoplasmic light chains showed a monoclonal lambda pattern in nonphagocytic myeloma cells, and a probable monoclonal lambda pattern in phagocytic myeloma cells. These findings characterize the neoplasm as a monoclonal proliferation of differentiated plasma cells with the capability of erythrophagocytosis. Erythrophagocytosis by myeloma cells may have been responsible for the hemolytic anemia. The tritiated thymidine labeling index (LI%) was high (8%), suggesting a poor prognosis, despite a dramatic initial response to chemotherapy.
一例噬血细胞性λ轻链浆细胞骨髓瘤，通过其临床、形态学、细胞化学、免疫学及细胞动力学特征得以明确。一名40岁男性患者，表现为抗人球蛋白试验阴性的溶血性贫血、肝脾肿大、溶骨性骨损害、λ轻链单克隆丙种球蛋白病，以及发育异常的浆细胞浸润骨髓，其中10%的浆细胞显示对红细胞有吞噬作用。电子显微镜检查显示骨髓瘤细胞有明显的细胞质微丝，且细胞质空泡内有红细胞。骨髓瘤细胞在体外不吞噬葡萄球菌。吞噬性和非吞噬性骨髓瘤细胞对酒石酸敏感、酸性磷酸酶阳性、α-萘丁酸酯酶阴性，不形成E玫瑰花结或EAox（IgG）玫瑰花结。肿瘤细胞末端脱氧核苷酸转移酶、Ia抗原及表面免疫球蛋白均为阴性。细胞质轻链的免疫荧光染色显示，非吞噬性骨髓瘤细胞呈单克隆λ型，吞噬性骨髓瘤细胞可能呈单克隆λ型。这些发现表明该肿瘤是具有红细胞吞噬能力的分化浆细胞的单克隆增殖。骨髓瘤细胞的红细胞吞噬作用可能是溶血性贫血的原因。尽管对化疗最初有显著反应，但氚标记胸腺嘧啶核苷标记指数（LI%）较高（8%），提示预后不良。
