Iwasaki T, Hamano T, Ogata A, Hashimoto N, Kakishita E
Second Department of Internal Medicine, Hyogo College of Medicine, Nishinomiya, Hyogo, Japan.
Acta Haematol. 2000;104(1):42-5. doi: 10.1159/000041069.
We present a unique case of IgD multiple myeloma (MM) preceding the development of extensive extramedullary disease without medullary involvement. A 63-year-old man was diagnosed with IgD-lambda MM when he developed anemia. After 3 months of chemotherapy, he was in complete remission as evidenced by the disappearance of bone marrow (BM) plasmacytosis, monoclonal IgD protein in his serum, and Bence Jones proteinuria. Six months after diagnosis, his disease took an unusual course with the development of plasmacytomas in the skin, without medullary involvement. He then received chemotherapy, resulting in the complete disappearance of the subcutaneous plasmacytomas. Two years after the initial diagnosis, his disease took an aggressive clinical course with retroperitoneal relapse, leading to the patient's death within 1 month. The two separate episodes of extramedullary disease were associated with elevated serum lactic dehydrogenase levels and the absence of plasma cells in the BM. This case provides evidence of two separate transformations of the original malignant MM clone.
我们报告了一例独特的IgD型多发性骨髓瘤（MM）病例，在广泛的髓外疾病发生之前未出现骨髓受累。一名63岁男性在出现贫血时被诊断为IgD-λ型MM。化疗3个月后，他达到完全缓解，骨髓浆细胞增多、血清中单克隆IgD蛋白及本周蛋白尿消失可证明。诊断6个月后，他的病情出现异常进展，皮肤出现浆细胞瘤，未累及骨髓。随后他接受了化疗，皮下浆细胞瘤完全消失。初始诊断两年后，他的病情呈侵袭性临床经过，出现腹膜后复发，导致患者在1个月内死亡。两次独立的髓外疾病发作均与血清乳酸脱氢酶水平升高及骨髓中无浆细胞有关。该病例提供了原始恶性MM克隆发生两次独立转变的证据。
