Outcomes of dose-adjusted Berlin-Frankfurt-Münster-90 regimen without radiotherapy in adolescents and adults with T cell lymphoblastic lymphoma.

Yan Xie,Yuqin Song,Xiaopei Wang,Lingyan Ping,Weiping Liu,Chen Zhang,Jun Zhu,Yuntao Zhang,Lijuan Deng,Ningjing Lin,Meifeng Tu,Zhitao Ying,Wen Zheng

doi:10.1007/s12032-015-0551-9

Abstract

The aim of this study was to evaluate the outcomes using the dose-adjusted Berlin–Frankfurt–Munster (BFM-90) regimen without radiotherapy in adolescents and adults with T cell lymphoblastic lymphoma (T-LBL) at Beijing Cancer Hospital. Between March 2004 and December 2013, 57 newly diagnosed T-LBL patients were treated in our center. We retrospectively analyzed their main clinical characteristics and prognosis. The media age of the patients at diagnosis was 26 (range 14–54). At a median follow-up of 24 months (range 5–119), 38 patients (67 %) were alive. The estimated 3-year overall survival (OS) rate and progression-free survival (PFS) rate were 64 and 60 %, respectively. Abnormal WBC at diagnosis, high IPI and no early response were indicated as adverse prognostic factors for both PFS and OS (p < 0.05). There was also a trend for better survival in autologous peripheral blood stem cell transplantation (APBSCT) group as compared to non-APBSCT group (3-year OS 83 vs. 57 %), but without any significant difference. This study suggested that the dose-adjusted BFM-90 protocol without irradiation showed comparable long-term results in Chinese adolescents and adults with T-LBL. APBSCT may become a choice whether we can identify the best candidate.

Highlights

T cell lymphoblastic lymphoma (T-LBL) and T cell acute lymphoblastic leukemia (T-ALL) are categorized as precursor T cell malignancy, frequently accompanied by a mediastinal mass and a high prevalence of central nervous system (CNS) involvement [1]
This study suggested that the dose-adjusted BFM-90 protocol without irradiation showed comparable long-term results in Chinese adolescents and adults with T-LBL
Because T-LBL cell marker expression overlaps that of T-ALL, the clinical distinction between the two entities is arbitrarily determined by the degree of bone marrow (BM) involvement: Patients with more than 25 % lymphoblasts are classified as having T-ALL, whereas those with a lesser degree of marrow replacement are classified as having T-LBL [2]

Summary

Introduction

T cell lymphoblastic lymphoma (T-LBL) and T cell acute lymphoblastic leukemia (T-ALL) are categorized as precursor T cell malignancy, frequently accompanied by a mediastinal mass and a high prevalence of central nervous system (CNS) involvement [1]. Because T-LBL cell marker expression overlaps that of T-ALL, the clinical distinction between the two entities is arbitrarily determined by the degree of bone marrow (BM) involvement: Patients with more than 25 % lymphoblasts are classified as having T-ALL, whereas those with a lesser degree of marrow replacement are classified as having T-LBL [2]. T cell lymphoblastic lymphoma/leukemia accounts for approximately 3.4 % of all non-Hodgkin lymphomas (NHLs) in China [3]. The prognosis of LBL has dramatically improved with the use of intensive ALL-type chemotherapy regimens, with an event-free survival (EFS) of 90 % in children and disease-free survival (DFS) of 72 % in adults [5, 6]. The use of ALL-type regimens such as BFM-90 regimen usually acquired better survival rates, they brought more adverse events as well as treatment-related death. The aim of this study was to evaluate the outcomes using the dose-adjusted Berlin– Frankfurt–Munster (BFM-90) regimen without radiotherapy in adolescents and adults in our cancer center

Objectives

Methods

Results

Conclusion