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Light Chain Escape Multiple Myeloma Complicated with Catastrophic Extramedullary Plasmacytoma Following Novel Agent Treatment

經多線新藥治療後多發性骨髓瘤產生之“輕鍊脫逃”現象及災難性髓外漿細胞瘤

摘要


多發性骨髓瘤的治療在近幾年來蓬勃的發展,使得病患的存活時間幾乎延長至兩倍,不過,存活時間的進步也導致一些原本不常見的臨床情況出現,“輕鏈脫逃”和“髓外漿細胞瘤”就是其中的代表。在這裡我們提出了一個多發性骨髓瘤的臨床案例,就是在接受了包含傳統化療、自體幹細胞移植、以及標靶藥物後同時發生了這兩種現象,這個病患的治療結果是令人失望的。但是我們從這個案例學到了一些經驗:第一、免疫球蛋白的檢查在多發性骨髓瘤的監控上可能不是一個可靠的工具,需要合併腎功能檢查、骨骼檢查、以及血漿游離輕鍊檢查,才能得到最好的監測效果;第二,髓外漿細胞瘤仍是一個非常難治療的病症,仍有賴後續的研究來發展出真正有效的治療藥物,以幫助更多的病患。

並列摘要


The treatment of multiple myeloma (MM) has made greatly advances over the last decade, nearly doubling the overall survival time after the use of novel agents such as thalidomide, lenalidomide and bortezomib. However, the improvement of overall survival has occasionally led to clinical scenarios that were rarely recognized before, for example, light chain escape and extramedullary plasmacytoma (EMP). Here we report an MM patient who relapsed and manifested simultaneously with both a light chain escape and a disseminated pattern of EMP after several different modes of treatment, including conventional chemotherapy, autologous peripheral blood stem cell (PBSC) transplantation, and novel agents. The result was disappointing in this case, which taught us several important things: First, intact immunoglobulin may not be a reliable marker in the surveillance of pretreated multiple myeloma. Various tests for renal function, skeletal condition, and serum free light chain should also be carried out for a comprehensive evaluation. Second, EMP remains a tremendous challenge to the clinician, especially when refractory to current novel agents. Further researches are required to clarify the optimal treatment of such a condition in order to help more patients.

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