van Tol K M, Fedder G
Locatie Lukas Ziekenhuis, afd. Inwendige Geneeskunde, Ziekenhuis Centrum Apeldoorn.
Ned Tijdschr Geneeskd. 1994 Mar 5;138(10):526-8.
Multiple skin lesions developed in a 70 year-old-man. Pathological examination revealed plasmacytomas. Because of the presence of a IgD paraprotein and 15% abnormal plasma cells in the bone marrow Kahler's disease of the IgD type was diagnosed. In accordance with literature the course of the disease was rapidly progressive. After an initial favourable response to chemotherapy and radiotherapy, the patient died one year after diagnosis. Extraosseous tissue involvement and hepatosplenomegaly are well-known phenomenon of IgD-Kahler's disease. Skin plasmacytomas as the first symptom of the disease are very rare.
一名70岁男性身上出现了多处皮肤病变。病理检查显示为浆细胞瘤。由于存在IgD副蛋白且骨髓中有15%的异常浆细胞,诊断为IgD型卡勒氏病。根据文献记载,该疾病的病程进展迅速。在对化疗和放疗最初有良好反应后,患者在诊断后一年死亡。骨外组织受累和肝脾肿大是IgD型卡勒氏病的常见现象。皮肤浆细胞瘤作为该疾病的首发症状非常罕见。