Long-term Survival in a Patient With Transformation of Waldenström’s Macroglobulinemia into DLBCL

医学 华登氏巨球蛋白血症 美罗华 弥漫性大B细胞淋巴瘤 淋巴浆细胞淋巴瘤 巨球蛋白血症 淋巴瘤 BCL6公司 化学免疫疗法 内科学 肿瘤科 胃肠病学 免疫学 B细胞 抗体 生发中心 多发性骨髓瘤
作者
Eirini Solia,Ioannis Ntanasis‐Stathopoulos,Efstathios Kastritis,Evangelos Terpos,Meletios Α. Dimopoulos,Maria Gavriatopoulou
出处
期刊:Cancer Diagnosis & Prognosis [Anticancer Research USA Inc.]
卷期号:4 (1): 77-80
标识
DOI:10.21873/cdp.10289
摘要

Background/Aim: Waldenström’s macroglobulinemia (WM) is a rare slow-growing B-cell lymphoma that is characterized by lymphoplasmacytic bone marrow infiltration and the production of monoclonal immunoglobulin M (IgM) paraprotein. In 5-10% of patients, WM undergoes transformation into diffuse large B-cell lymphoma (DLBCL), which is more aggressive, with poor prognosis and a low survival rate. Case Report: Α 69-year-old woman was diagnosed with WM in 2009. She received six cycles of chemoimmunotherapy and a remarkable remission was achieved. However, in 2013 the disease transformed into DLBCL. The patient received chemotherapy and after the completion of the first cycle of therapy, the disease was significantly minimized. At the end of the therapy, there was no evidence of disease, and the patient remains disease-free. The cytogenetic profile of the patient did not reveal expression of BCL2 apoptosis regulator, BCL6 transcription repressor, Epstein–Barr virus small RNA, syndecan 1 nor cyclin D1. According to a staging system based on the platelet count, lactate dehydrogenase and previous treatment for WM, the described patient was classified as being at intermediate risk with an expected 2-year survival probability of 47% after WM transformation into DLBCL. However, the patient unexpectedly exceeded these prognostic indications. Conclusion: The findings for this patient are of great interest compared with the existing literature which suggests that the survival and prognosis for patients with transformed DLBCL are not favorable.
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