PDF(Pubmed)
Abstract：
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
摘要：
■Waldenström的巨球蛋白血症（WM）是一种罕见的缓慢生长的B细胞淋巴瘤，其特征是淋巴浆细胞性骨髓浸润和单克隆免疫球蛋白M（IgM）副蛋白的产生。在5-10%的患者中，WM转化为弥漫性大B细胞淋巴瘤（DLBCL），更具侵略性，预后差，生存率低。
■A69岁的女性在2009年被诊断为WM。她接受了六个周期的化学免疫疗法，并获得了显着的缓解。然而,2013年，该病转化为DLBCL。患者接受化疗，第一周期治疗结束后，疾病显著减少。在治疗结束时,没有疾病的证据,病人仍然没有疾病。患者的细胞遗传学图谱未显示BCL2凋亡调节因子的表达，BCL6转录阻遏物，EB病毒小RNA,syndecan1或cyclinD1。根据基于血小板计数的分期系统，乳酸脱氢酶和以前的WM治疗，在WM转化为DLBCL后,所述患者被分类为中危,预期2年生存概率为47%.然而,患者意外地超过了这些预后指征.
■与现有文献相比，该患者的发现引起了极大的兴趣，现有文献表明，转化的DLBCL患者的生存和预后不佳。
■A69岁的女性在2009年被诊断为WM。她接受了六个周期的化学免疫疗法，并获得了显着的缓解。然而,2013年，该病转化为DLBCL。患者接受化疗，第一周期治疗结束后，疾病显著减少。在治疗结束时,没有疾病的证据,病人仍然没有疾病。患者的细胞遗传学图谱未显示BCL2凋亡调节因子的表达，BCL6转录阻遏物，EB病毒小RNA,syndecan1或cyclinD1。根据基于血小板计数的分期系统，乳酸脱氢酶和以前的WM治疗，在WM转化为DLBCL后,所述患者被分类为中危,预期2年生存概率为47%.然而,患者意外地超过了这些预后指征.
■与现有文献相比，该患者的发现引起了极大的兴趣，现有文献表明，转化的DLBCL患者的生存和预后不佳。
