Stage, age, and EBV status impact outcomes of plasmablastic lymphoma patients: a clinicopathologic analysis of 61 patients.

Stage, age, and EBV status impact outcomes of plasmablastic lymphoma patients: a clinicopathologic analysis of 61 patients.
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DOI:
10.1186/s13045-015-0163-z
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发表时间:
2015-06-10
影响因子:
28.5
通讯作者:
Khoury JD
Khoury JD
中科院分区:
医学1区
文献类型:
--
作者:
Loghavi S;Alayed K;Aladily TN;Zuo Z;Ng SB;Tang G;Hu S;Yin CC;Miranda RN;Medeiros LJ;Khoury JD

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浆母细胞淋巴瘤(PBL)是一种罕见的侵袭性肿瘤,具有淋巴细胞和浆细胞分化,通常与免疫缺陷和预后不良有关。临床病理特征主要来源于病例报告和结果分析有限的小系列。对61例PBL患者的人口学、临床病理特征和临床结果进行了回顾和分析。患者的中位年龄为49岁(21-83岁),大多数(49/61;80%)为男性。50名患者有人类免疫缺陷病毒(艾滋病毒)状况:20名艾滋病毒阳性,30名艾滋病毒阴性。23例患者免疫功能正常。47例患者中有14例(30%)出现腹部/胃肠道不适,是最常见的症状。在就诊时,43例患者中有24例(56%)为III期或IV期疾病。57例中有40例(70%)检出eb病毒。在评估的10/15例(67%)病例中发现MYC重排,无论MYC重排状态如何,所有评估的病例中都发现MYC过表达。hiv阳性患者明显比hiv阴性患者年轻(中位42岁vs. 58岁;p = 0.006)。与hiv阴性患者相比,hiv阳性患者发生ebv阳性疾病的可能性也显著增加(19/ 19,100% vs. 15/ 29,52%; p = 0.002)。与接受高分割环磷酰胺、长春新碱、阿霉素和地塞米松(hyper-CVAD)化疗的患者相比,接受CHOP化疗的患者往往有更好的总生存期(OS) (p = 0.078)。HIV感染状况对OS没有影响。ebv阳性PBL患者的无事件生存期(EFS)较好(p = 0.047),但OS较差(p = 0.306)。值得注意的是,年龄≥50岁(p = 0.013)、III期或IV期疾病(p = <0.001)和淋巴结受累(p = 0.008)对OS有不利影响。PBL患者最重要的预后参数是年龄、分期,其次是EBV状态。在这项研究中,评估的PBL病例中有三分之二与MYC重排有关,并且均显示MYC过表达。本文的在线版本(doi:10.1186/s13045-015-0163-z)包含补充资料,仅供授权用户使用。
Plasmablastic lymphoma (PBL) is a rare aggressive neoplasm with lymphoid and plasmacytic differentiation that is commonly associated with immunodeficiency and an unfavorable prognosis. Clinicopathologic features have been largely derived from cases reports and small series with limited outcome analyses. The demographic, clinicopathologic features, and clinical outcomes of a cohort of 61 patients with PBL were reviewed and analyzed. Patients had a median age of 49 years (range 21–83 years) and most (49/61; 80 %) were men. Human immunodeficiency virus (HIV) status was available for 50 patients: 20 were HIV-positive and 30 HIV-negative. Twenty-three patients were immunocompetent. Abdominal/gastrointestinal complaints were the most common presenting symptoms, reported in 14 of 47 (30 %) of patients. At presentation, 24 of 43 (56 %) patients had stage III or IV disease. Epstein-Barr virus (EBV) was detected in 40 of 57 (70 %) cases. MYC rearrangement was identified in 10/15 (67 %) cases assessed, and MYC overexpression was seen in all cases assessed regardless of MYC rearrangement status. HIV-positive patients were significantly younger than those who were HIV-negative (median 42 vs. 58 years; p = 0.006). HIV-positive patients were also significantly more likely to have EBV-positive disease compared with HIV-negative patients (19/19, 100 % vs. 15/29, 52 %; p = 0.002). Patients who received CHOP chemotherapy tended to have better overall survival (OS) compared with those who received hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone (hyper-CVAD) (p = 0.078). HIV status had no impact on OS. Patients with EBV-positive PBL had a better event-free survival (EFS) (p = 0.047) but not OS (p = 0.306). Notably, OS was adversely impacted by age ≥50 years (p = 0.013), stage III or IV disease (p = <0.001), and lymph node involvement (p = 0.008). The most significant prognostic parameters in patients with PBL are age, stage, and, to a lesser extent, EBV status. In this study, two-thirds of PBL cases assessed were associated with MYC rearrangement and all showed MYC overexpression. The online version of this article (doi:10.1186/s13045-015-0163-z) contains supplementary material, which is available to authorized users.
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