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題名 | Immunoglobulin D Multiple Myeloma=免疫球蛋白D型多發性骨髓瘤 |
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作者姓名(中文) | 林棟樑; 施麗雲; 鄧波; 王博南; 吳金和; 郭明宗; | 書刊名 | 長庚醫學 |
卷期 | 23:8 2000.08[民89.08] |
頁次 | 頁451-457 |
分類號 | 416.66 |
關鍵詞 | 多發性骨髓瘤; 免疫球蛋白D; 存活期; Multiple myeloma; Immunoglobulin D; Survival; |
語文 | 英文(English) |
英文摘要 | BACKGROUND: Immunoglobulin D (IgD) multiple myeloma (MM) is rare, accounting for less than 2% of all patients with MM in Western countries. In Taiwan, the frequency and clinicopathologic features of IgD MM have not yet been reported. METHODS: The clinicopathologic features and treatment outcome of patients with IgD MM diagnosed between January 1, 1982, and December 31, 1998, in Chang Gung Memorial Hospital were retrospectively reviewed. Nineteen patients with IgD MM were diagnosed. Of those patients, the medical records of 16 were available for review. RESULTS: Most of the patients were male (11/16) with a median age of 59 years. The most common presenting features included bone pain (56%), gastrointestinal discomfort (38%), general malaise (38%), and body weight loss (25%). The majority of patients had cytopenia (88%), renal function impairment (75%), hypercalcemia (63%), hyperuricemia (69%), elevated β2-microglobulin levels (86%), and Bence Jones proteinuria (92%). Serum protein electrophoresis showed an M-peak in 9 cases (9/12), whereas immunoelectrophoresis or immunofixation identified an IgD monoclonal gammopathy in all 16 patients. All of the M-proteins were of a lambda-light chain type. The IgD level ranged between 584 and 129,000 IU/ml (normal, <100 IU/ml). All the patients had stage III disease except one (stage I). Four patients were still alive at the time of this analysis. The median survival time was 12 months. Infection was the leading cause of death (50%). CONCLUSION: The present series showed that IgD MM had aggressive clinical features, male predominance, a high frequency of renal function impairment, high incidence of M-protein undetected by serum protein electrophoresis, a predilection for lambdalight chains, and a short period of survival. |
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