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Four hundred and sixty-seven hematopoietic stem cell transplantations (HSCTs) (217 autologous and 250 allogeneic HSCT) were performed in 374 children at four pediatric HSCT centers in Korea from January 2005 to December 2007. Among 467 transplants, veno-occlusive disease (VOD) developed in 72 transplants (15.4%) at a median of 10 days after HSCT. Multivariate analysis showed that BU or TBI-containing regimen (P ¼ 0.002), VOD prophylaxis without lipo-prostaglandin E1 (PGE1) (P ¼ 0.012), number of previous HSCT (P ¼ 0.014), and pretransplant serum ferritin (P ¼ 0.018) were independent risk factors for developing VOD. Mean serum ferritin levels were significantly higher in HSCT with VOD (2109.6 ± 2842.5 ng/ml) than in HSCT without VOD (1315.9±1094.4 ng/ml) (Po0.001). The relative risk of death within 100 days of HSCT in transplants with VOD compared with transplants without VOD was 3.39 (confidence interval: 1.78-6.45). Our results suggest that lipo-PGE1 might have a protective effect against the development of VOD, and pretransplant serum ferritin could act as a risk factor for VOD. A larger prospective study is needed to confirm a possible role of lipo-PGE1 and iron chelation therapy in reducing the incidence of VOD.

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High-dose chemotherapy and autologous stem cell transplantation (ASCT) for extranodal natural killer/T-cell lymphoma (ENKTL) is a reasonable option for a subset of patients. The impact of response status, according to positron emission tomography/computed tomography (PET/CT) results and/or presence of circulating EBV DNA prior to ASCT, has not yet been established. We analyzed 27 ENKTL patients with pre-ASCT circulating EBV DNA who had undergone pre-ASCT PET/CT between 2009 and 2014. We classified patients into two groups based on the result of pretransplantation assessment: a favorable risk group (pretransplant five-point Deauville score (DS) of 1-2 based on PET/CT and no detectable EBV DNA) and an unfavorable risk group (DS 1-2 with detectable EBV DNA, DS 3-5 with or without detectable EBV DNA). After a median follow-up of 37 months, overall survival and PFS were significantly different between the two groups (median OS: not reached for favorable risk group vs 7.0 months for unfavorable risk group, P = 0.017; median PFS: 16.0 vs 5.0 months, P = 0.019). Multivariate analysis revealed that pre-ASCT DS and EBV DNA was the only independent prognostic factor considering stage, IPI and NKPI. Precise assessment of the status of disease before transplantation may provide more benefit from ASCT to ENKTL patients.Bone Marrow Transplantation (2016) 51, 807-812; doi:10.1038/bmt.2016.6; published online 8 February 2016 INTRODUCTIONExtranodal natural killer/T-cell lymphoma (ENKTL) is a rare and distinct subtype of non-Hodgkin lymphoma (NHL), which is characterized by clinically aggressive behavior leading to poor survival among all T-cell lymphoma subtypes. 1 Concurrent chemoradiotherapy (CCRT) followed by chemotherapy has shown promising results with manageable toxicities for localized disease, 2,3 and the application of nonanthracycline-based chemotherapy incorporating L-asparaginase has improved survival outcome of advanced, relapsed or refractory ENKTL patients. [4][5][6] However, treatment failure still occurs frequently, and thus the unmet need persists for additional therapies to improve outcomes for patients with ENKTL. Accordingly, highdose chemotherapy with autologous stem cell transplantation (ASCT) has been evaluated as a consolidation therapy for ENKTL. However, previous studies have failed to clearly determine the efficacy of ASCT, because most of them were retrospective analyses with small sample sizes, and the enrolled patient populations were very heterogeneous, including various transplantation settings. 7-11 Moreover, although previous data showed that disease status at the time of ASCT was the most important prognostic factor for survival and relapse-free survival, 9,11 the optimal response assessment with respect to existing computed tomography (CT) scans, positron emission tomography/computed tomography (PET/CT) findings, and EBV

In an effort to improve survival and reduce late adverse effects of radiation therapy (RT), 25 children o3 years of age with malignant brain tumors received tandem high-dose chemotherapy (HDCT) and auto-SCT following six cycles of induction chemotherapy. RT was either not given or deferred until 3 years of age if the patient was in CR after tandem HDCT/auto-SCT. Tumors relapsed or progressed in nine patients (five during induction treatment), and two of these patients survived after receiving salvage treatment, including RT. Two patients died due to toxicities during tandem HDCT/auto-SCT. A total of 16 patients survived to a median follow-up period of 52 months (range 18-96) from the time of diagnosis. Four of these patients did not receive RT, two received local RT (L-RT), three received craniospinal RT (CSRT), and seven received both L-RT and CSRT. The 5-year OS and EFS rates were 67.8 ± 9.4% and 55.5 ± 10.0%, respectively. Neuroendocrine and neurocognitive functions evaluated 3 years after tandem HDCT/auto-SCT were acceptable. Our results indicate that tandem HDCT/auto-SCT may improve survival in young children with malignant brain tumors with an acceptable level of risk of long-term toxicity.

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