Diffuse large B-cell lymphoma occurring in patients with lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia - Clinicopathologic features of 12 cases
Diffuse large B-cell lymphoma occurring in patients with lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia - Clinicopathologic features of 12 cases
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DOI：
10.1039/r01vxg46mfcdvnhl
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发表时间：
2003-08-01
影响因子：
3.5
通讯作者：
Medeiros, LJ
中科院分区：
文献类型：
--
作者：
Lin, P;Mansoor, A;Medeiros, LJ
在我们机构治疗的92例淋巴浆细胞性淋巴瘤/瓦尔登斯特伦巨球蛋白血症（LPL/WM）患者中，12例（13%）还发生了弥漫性大B细胞淋巴瘤（DLBCL）。在10例患者中，DLBCL在诊断LPL/WM后12至128个月（中位数，44个月）发生。2例患者同时患有LPL/WM和DLBCL。诊断LPL/WM时的临床病理特征不能预测DLBCL的风险。DLBCL的发作特征为全身症状恶化、严重血细胞减少、髓外疾病和器官肿大。LPL/WM和DLBCL中免疫球蛋白轻链表达相同。8例DLBCL中EB病毒（EBV）原位杂交均为阴性。在11例有临床随访信息的患者中，8例（73%）在诊断DLBCL后10个月内死亡。DLBCL最可能是组织学转化的结果，发生在LPL/WM患者的一个子集中，与侵袭性临床病程和不良结局相关。EBV不太可能参与转化。
Of 92 patients with lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia (LPL/WM) treated at our institution, diffuse large B-cell lymphoma (DLBCL) also developed in 12 (13%). In 10 patients, DLBCL developed 12 to 128 months (median, 44 months) after the diagnosis of LPL/WM. Two patients had LPL/WM and DLBCL simultaneously. Clinicopathologic features at diagnosis of LPL/WM did not predict the risk of DLBCL. Onset of DLBCL was characterized by worsening constitutional symptoms, profound cytopenias, extramedullary disease, and organomegaly. Immunoglobulin light chain expression was identical in both LPL/WM and DLBCL. In situ hybridization for Epstein-Barr virus (EBV) in 8 cases of DLBCL was negative. Of 11 patients with clinical follow-up information available, 8 (73%) died within 10 months of diagnosis of DLBCL. DLBCL, most likely as a result of histologic transformation, occurs in a subset of patients with LPL/WM and is associated with aggressive clinical course and poor outcome. EBV is unlikely to be involved in transformation.