期刊论文详细信息
Journal of Hematology & Oncology
Stage, age, and EBV status impact outcomes of plasmablastic lymphoma patients: a clinicopathologic analysis of 61 patients
Joseph D. Khoury2  L. Jeffrey Medeiros2  Roberto N. Miranda2  C. Cameron Yin2  Shimin Hu2  Guilin Tang2  Siok-Bian Ng1  Zhuang Zuo2  Tariq N. Aladily3  Khaled Alayed4  Sanam Loghavi2 
[1] Department of Pathology, National University of Singapore and Cancer Science Institute, Singapore, Singapore;Department of Hematopathology, The University of Texas, M.D. Anderson Cancer Center, 1515 Holcombe Boulevard, MS-072, Houston 77030, TX, USA;Department of Pathology and Laboratory Medicine, The University of Jordan, Amman, Jordan;Department of Pathology, King Saud University, Riyadh, Saudi Arabia
关键词: MYC;    Immunocompetent;    Immunodeficiency;    HIV;    EBV;    Plasmablastic lymphoma;   
Others  :  1217371
DOI  :  10.1186/s13045-015-0163-z
 received in 2015-02-27, accepted in 2015-05-28,  发布年份 2015
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【 摘 要 】

Background

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.

Patients and methods

The demographic, clinicopathologic features, and clinical outcomes of a cohort of 61 patients with PBL were reviewed and analyzed.

Results

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).

Conclusions

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.

【 授权许可】

   
2015 Loghavi et al.

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