Efficacy Of Modified NHL BFM-95Regimen On Children With Precursor T Cell Lymphoblastic Lymphoma

Precursor T cell lymphoblastic lymphoma (pre T-LBL), also known as T celllymphoblastic lymphoma (T-LBL), originated in the thymus lymphoid tissue.Compared to T-cell acute lymphoblastic leukemia (T-ALL), it is a varied clinicalsubtype of a disease with similar features on histology, immune phenotype andcytogenetic characteristics. On histology, the former is more mature than the latter,which majorly presents with local mass and bone marrow involvement in seriouscases. Under the current treatment outcome, in children, overall survival rate is above90%in children with pre T-LBL. Among children ages1~9years, event-free survivalrate is the highest. At present, the uesed-commonly regimen originated from fromEurope BFM (Berlin-Frankfurt-Munster) organization. The treatment withhigh-dose methotrexate (HD-MTX) is an important during consolidation.Objective: To evaluate the efficacy of Modified NHL BFM-95Regimen (HD-dose MTX5.0g/m2reduced to3.0g/m2) on children with precursor T celllymphoblastic lymphoma.Methods: On histopathologic diagnosis,34children with pre T-LBL wereenrolled into this clinical trial, whom were treated with modified NHL BFM-95regimen from September2008and June2011. The patient’s characteristic, treatementresponse (d33), treatment outcome (event-free survival, total overall survival) andincidences of toxicities were respectively analyzed, compared with the original NHL-BFM-95(methotrexate5g/m2).Results: Of34pre T-LBL patients, male28(82.3%), female6(17.7%), themedian age was8.3year old(19month～15year old) at the diagnosis. The medianfollow-up was3years (1.5~4.5years). Evaluations on day33revealed that31children (91.2%) achieved CR, PR in2children (8.8%), and the response rate was 100%. Through the induction therapy,23children (67.6%) finished it≥75days,while11children (32.4%)＜75days.During consolidation therapy,7children （20.6%） suffered frommyelosuppression,11children （32.4%） mucositis,8children （23.5%）gastrointestinal tract reaction,8children（23.5%）liver damage,6children （17.6%）febrile neutropenia. Compared to the original NHL BFM-95outcomes, the childrenin this clinical trial benefited from the modified NHL BFM-95regimen onchemotherapy-related complications, including the incidence of mucositis,gastrointestinal tract reaction, bone marrow suppression, and liver function injury,the benefits remained statistically significant.After the chemotherapy, the overall survival rate was88.2%in the group ofchildren, the disease-free survival rate was85.3%. The OS of Stage Ⅲ and Ⅳ OSwere90%and86.4%respectively, EFS were90%and81.8%respectively.Compared to the original NHL BFM–95, on the clinical features, there was nostatistically significant difference. Of the34patients,5children (14.7%) experiencedrecurrence, of whom4cases（80%）died because of disease progression becauseof giving up further treatment,1case (20%) survived after receiving high-risk ALLchemotherapy regimen. Among them,1child’s recurrent site were bone marrow andtesticle, while the rest were bone marrows; there was no case presentinglocal recurrence or/and central nervous system relapse. Recurrence time range was6.4months~13months after chemotherapy. In recurrent children,4cases (80%)presented CR,1case (20%) PR on day33evaluation.Conclusion: Compared with the original BFM-95regimen, themodified BFM-95regimen can effectively treat children with pre T-NHL, but itremains to be confirmed by clinical multi-center randomized controlled study, whichsignificantly reduced incidences of some complications related to the usage ofhigh-dose methotrexate. The length of delay time during induction therapy in preT-NHL children may be one of the factors affecting the prognosis.