2026-07-11 | HSCT May Improve Survival in Advanced Extranodal NK/T-Cell Lymphoma: A Single-Center Retrospective Study.
Extranodal NK/T-cell lymphoma (ENKTCL) is a rare and aggressive lymphoid malignancy highly associated with Epstein-Barr virus (EBV) infection. Patients with stage III or higher disease often have a poor prognosis. This study retrospectively reviewed the treatment courses of 34 patients with relapsed/refractory (R/R) ENKTCL who underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) at our center between January 2019 and July 2024. The results showed that in this study, the use of PD-1 inhibitors before transplantation, the number of treatment lines, and whether the plasma EBV was negative or not had no significant impact on OS and PFS. Patients achieving complete response (CR) or partial response (PR) prior to allo-HSCT exhibited a more favorable prognosis. Conversely, a history of chronic active EBV infection (CAEBV) before transplantation and EBV reactivation post-transplantation were associated with inferior survival, suggesting poor prognostic outcomes. Genetic susceptibility analysis revealed that patients with advanced ENKTCL frequently harbored hemophagocytic lymphohistiocytosis (HLH)-associated gene variants. LYST gene variants were the most commonly identified HLH-related alterations, and patients developing HLH experienced significantly higher non-relapse mortality (NRM). Furthermore, TP53 gene variants were associated with post-allo-HSCT relapse. In conclusion, Allogeneic HSCT may improve survival in selected high-risk, advanced-stage R/R ENKTCL patients. These single-center, retrospective findings are hypothesis-generating and require validation in larger, multi-center studies with longer follow-up.
Open article ↗
2026-07-09 | Pediatric extranasal, EBV-negative, extranodal natural killer/T-cell lymphoma; case report.
Extranodal natural killer/T-cell lymphomas (ENKTCL) are rare, aggressive neoplasms primarily occurring in adults of Asian or Native American descent. Most are associated with Epstein-Barr virus (EBV) and originate in the nasopharyngeal region. We present the case of a fulminant, disseminated, non-nasal, EBV negative, seemingly de novo ENKTCL in an infant who presented with a five-day history of progressive abdominal distention, respiratory distress, irritability, non-bilious emesis, right eye proptosis, and pallor. Initial labs revealed leukocytosis, anemia and thrombocytopenia. Computerized axial tomography revealed densities in the right optic nerve, superior orbits, thymus, posterior mediastinum, pericardium, myocardium, axillary lymph nodes, adrenal glands, kidneys and pancreas. The patient rapidly deteriorated, and despite extensive resuscitation, ultimately died. Autopsy revealed tumors in many organs, displaying vascular damage and tissue necrosis with lymphoid infiltration. The likely diagnosis of EBV-negative ENKTCL was confirmed after further histologic analysis. Pediatric ENKTCL is a particularly uncommon tumor, especially given our patient's very young age and EBV-negative status. Determining the best treatment regimen for these children is challenging due to limited data. Treatment typically consists of combination chemotherapy and radiation, but targeted therapies are being explored with promising results.
Open article ↗
2026-07-07 | Locoregional recurrence patterns and prognostic outcomes in early-stage natural killer/T-cell lymphoma treated with sandwich chemoradiotherapy: A post hoc analysis of a randomized controlled trial.
This study aimed to evaluate the locoregional control in early-stage natural killer/T-cell lymphoma (NKTCL) patients treated with sandwich chemoradiotherapy. We performed a post hoc analysis of 87 consecutive early-stage NKTCL patients enrolled in a prospective, multicenter, randomized phase III trial. All participants received standardized treatment comprising four cycles of asparaginase-based chemotherapy with integrated radiotherapy. With a median follow-up of 37.9 months, the 3-year survival outcomes of the cohort were: overall survival (OS) 87.1%, progression-free survival (PFS) 84.6%, and locoregional recurrence-free survival (LRRFS) 90.8%. Six patients experienced locoregional recurrence, with 4 in nasal cavity (4.6%), 4 in nasopharynx (4.6%), and 1 in cervical lymph node. Recurrence is typically in radiation field (5/6), often in the initial high-SUV region (4/6). Radiotherapy-chemotherapy interval (RCI) between 32-35 days was associated with improved prognosis, including OS (P = 0.043), PFS (P = 0.013), and LRRFS (P = 0.007) versus shorter RCI (21-31 days). Quantitative imaging analysis demonstrated that higher mean baseline apparent diffusion coefficient (ADC) values (≥0.1125 × 10⁻³mm²/s) was associated with an improved OS (95.2% vs 71.6%, P = 0.049). Asparaginase-based sandwich chemoradiotherapy shows favorable efficacy in early-stage NKTCL, with distinct locoregional recurrence patterns. RCI and ADC values emerge as critical prognostic factors, potentially informing treatment optimization.
Open article ↗
