Outcomes of doseadjusted Berlin–Frankfurt–Münster

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

Publish Date: 2015/03/10

Volume: 32, Issue: 4, Pages: 110-

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Abstract

The aim of this study was to evaluate the outcomes using the doseadjusted Berlin–Frankfurt–Munster BFM90 regimen without radiotherapy in adolescents and adults with T cell lymphoblastic lymphoma TLBL at Beijing Cancer Hospital Between March 2004 and December 2013 57 newly diagnosed TLBL 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 followup of 24 months range 5–119 38 patients 67  were alive The estimated 3year overall survival OS rate and progressionfree 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  005 There was also a trend for better survival in autologous peripheral blood stem cell transplantation APBSCT group as compared to nonAPBSCT group 3year OS 83 vs 57  but without any significant difference This study suggested that the doseadjusted BFM90 protocol without irradiation showed comparable longterm results in Chinese adolescents and adults with TLBL APBSCT may become a choice whether we can identify the best candidateT cell lymphoblastic lymphoma TLBL and T cell acute lymphoblastic leukemia TALL 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 TLBL cell marker expression overlaps that of TALL 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 TALL whereas those with a lesser degree of marrow replacement are classified as having TLBL 2 T cell lymphoblastic lymphoma/leukemia accounts for approximately 34  of all nonHodgkin lymphomas NHLs in China 3 The conventional CHOPlike regimen produced low complete remission CR rate17  and short media overall survival OS 85 months 4 But the prognosis of LBL has dramatically improved with the use of intensive ALLtype chemotherapy regimens with an eventfree survival EFS of 90  in children and diseasefree survival DFS of 72  in adults 5 6 Although the use of ALLtype regimens such as BFM90 regimen usually acquired better survival rates they brought more adverse events as well as treatmentrelated death The aim of this study was to evaluate the outcomes using the doseadjusted Berlin–Frankfurt–Munster BFM90 regimen without radiotherapy in adolescents and adults in our cancer centerBetween March 2004 and December 2013 patients not less than 14 years old with untreated TLBL were retrospectively analyzed All cases were pathologically diagnosed by biopsy material from a lymph node or tumor mass according to WHO classification of hematological malignancies in Beijing Cancer Hospital A series of markers including CD1a CD3 CD4 CD8 CD10 CD20 CD45RO CD79a Ki67 and terminal deoxynucleotidyl transferase TdT were performed by immunohistochemistryA complete panel was performed at the time of baseline staging restaging and final evaluation This panel included a full clinical history physical examination complete hematological and biochemical tests computed tomography CT scans of neck chest abdomen and pelvic or 18Ffluorodeoxyglucose positron emission tomography 18 FFDGPET if possible cerebrospinal fluid examination BM aspirate and/or biopsy Staging was carried out according to the Ann Arbor system for NHL Diagnosis of central nervous system CNS disease was made in case of blast cells found in the cerebrospinal fluid CSF or cerebral infiltration on cranial CT It is important to note that patients who had more than 25  blasts in the BM but presented as a bulky tumor were also included in this study

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