Hirayama Yasuo, Sakamaki Sumio, Tsuji Yasushi, Sagawa Tamotsu, Chiba Hiroki, Matsunaga Takuya, Kato Junji, Niitsu Yoshiro
Department of Internal Medicine, Higashi Sapporo Hospital.
Rinsho Ketsueki. 2003 Sep;44(9):957-61.
A 70-year-old woman was admitted for anemia, elevated serum total protein and a right axillary mass. Laboratory data showed monoclonal x IgM with a decrease in serum IgG and IgA levels. An occipital punched-out lesion was detected on a cranial X-ray. A tumor lesion was detected on chest X-ray and computed tomography. Biopsy specimen revealed plasmacytoma with cytoplasmic IgM. Bone marrow aspiration revealed an elevated plasma cell count. An immunophenotype analysis of the plasma cells showed positivity of cytoplasmic IgM, x, CD5, CD38, CD11a (LFA-1), CD44 (HCAM), CD49d (VLA-4) and CD54 (ICAM-1). From the above results, we diagnosed the patient as having IgM myeloma associated with plasmacytoma. Melphalan and prednisolone therapy were prescribed, their effect on the myeloma was short term, so we changed the chemotherapy to VAD (vincristine, adriamycin and dexamethasone), but this treatment had little effect. The patient developed bacterial pneumonia and died. IgM myeloma is a rare disease and reports of immunophenotype analysis are also rare. There is no case report of plasmacytoma associated with IgM myeloma.
一名70岁女性因贫血、血清总蛋白升高及右腋窝肿块入院。实验室检查数据显示单克隆x IgM，血清IgG和IgA水平降低。头颅X线检查发现枕部有穿凿样病变。胸部X线和计算机断层扫描发现肿瘤病变。活检标本显示为伴有细胞质IgM的浆细胞瘤。骨髓穿刺显示浆细胞计数升高。对浆细胞进行免疫表型分析显示细胞质IgM、x、CD5、CD38、CD11a（淋巴细胞功能相关抗原-1）、CD44（透明质酸介导的细胞黏附分子）、CD-49d（极迟抗原-4）和CD54（细胞间黏附分子-1）呈阳性。根据上述结果，我们诊断该患者患有与浆细胞瘤相关的IgM型骨髓瘤。给予美法仑和泼尼松治疗，其对骨髓瘤的疗效是短期的，因此我们将化疗方案改为VAD（长春新碱、阿霉素和地塞米松），但该治疗效果甚微。患者并发细菌性肺炎并死亡。IgM型骨髓瘤是一种罕见疾病，关于免疫表型分析的报道也很少。目前尚无与IgM型骨髓瘤相关的浆细胞瘤病例报告。
