Dierlamm T, Laack E, Dierlamm J, Fiedler W, Hossfeld D-K
University Hospital Hamburg-Eppendorf, Department of Oncology and Hematology, Martinistrasse 52, 20246 Hamburg, Germany.
Ann Hematol. 2002 Mar;81(3):136-9. doi: 10.1007/s00277-001-0420-9. Epub 2002 Feb 16.
IgM myeloma is a rare disease, accounting for approximately 0.5% of multiple myelomas (MM). Here we report four cases of IgM multiple myeloma. Two were diagnosed in advanced clinical stages with multiple osteolytic lesions, leading to hypercalcemia in one patient. Bone marrow morphology showed a variable degree of infiltration with mainly mature plasma cells. An immunophenotypic analysis performed in one case showed expression of CD38 and monoclonal cytoplasmatic immunoglobulin. Interphase fluorescence in situ hybridization performed in one case did not reveal any aneuploidies or deletions of the retinoblastoma, P16, or P53 tumor suppressor genes. While one patient with a smoldering IgM myeloma did not need specific therapy, the others received cytotoxic treatment based on standard chemotherapy for MM. The outcomes were one stable disease, one sustained complete remission, and one progressive disease. All four patients were alive 1 year after diagnosis. One died due to progressive disease after 31 months. We conclude that IgM myeloma shares clinical and histological features with other MM rather than with Waldenström's macroglobulinemia, which is most commonly diagnosed in cases with IgM monoclonal gammopathy. Since MM and Waldenström's macroglobulinemia differ in prognosis and treatment strategies, the two disease entities should be distinguished based on clinical criteria, bone marrow morphology, and immunophenotypic analysis.
IgM 骨髓瘤是一种罕见疾病，约占多发性骨髓瘤（MM）的0.5%。在此，我们报告4例IgM多发性骨髓瘤病例。其中2例在临床晚期被诊断，伴有多发溶骨性病变，1例患者出现高钙血症。骨髓形态学显示主要为成熟浆细胞的不同程度浸润。对1例病例进行的免疫表型分析显示CD38和单克隆细胞质免疫球蛋白表达。对1例病例进行的间期荧光原位杂交未发现视网膜母细胞瘤、P16或P53肿瘤抑制基因的任何非整倍体或缺失。1例冒烟型IgM骨髓瘤患者无需特殊治疗，其他患者接受基于MM标准化疗的细胞毒性治疗。结果为1例病情稳定，1例持续完全缓解，1例病情进展。所有4例患者在诊断后1年时均存活。1例在31个月后因病情进展死亡。我们得出结论，IgM骨髓瘤与其他MM具有共同的临床和组织学特征，而非与华氏巨球蛋白血症相同，华氏巨球蛋白血症最常见于IgM单克隆丙种球蛋白病病例。由于MM和华氏巨球蛋白血症在预后和治疗策略上存在差异，应根据临床标准、骨髓形态学和免疫表型分析对这两种疾病实体进行区分。
