Abstract
A broadly accepted standard treatment for adult T-lymphoblastic lymphoma (T-LBL) has not yet been defined. To address that issue, we retrospectively compared three chemotherapy regimens used to treat 110 adult patients with newly diagnosed T-LBL. These included two adult regimens (ECOG2993 and hyper-CVAD) and a childhood regimen (BFM-90). These intensive drug regimens are mainly used to treat childhood and adult acute lymphoblastic leukemia. They included induction, consolidation, and maintenance chemotherapy protocols and were administered over the course of 2 years. Seventy-five patients (80%) achieved a complete remission (CR). Within a median follow-up time of 31 months (range: 5–152 months), the 5-year overall survival (OS) and progression-free survival (PFS) rates were 47.7% (95% CI, 35.0–69.8%) and 45.7% (95% CI, 27.6–56.6%), respectively. Shorter survival was associated with age > 40 years, poor ECOG PS and bone marrow involvement. Elevated lactic dehydrogenase (LDH) level, Ann Arbor stage and International Prognostic Index (IPI) score had no prognostic value. The childhood chemotherapy regimen improved CR and the overall survival rate more than the adult regimen in patients aged < 40 years.
Highlights
T-lymphoblastic lymphoma (T-LBL) represents less than 2% of adult non-Hodgkin lymphomas (NHLs), but accounts for 85% to 90% of all lymphoblastic lymphomas [1]
The comparative baseline characteristics of all patients with respect to administration of childhood and adult regimens are summarized in Supplementary Table 1
For T-LBL patients, it appears that the adult acute lymphoblastic leukemia (ALL) chemotherapy regimen always produced poorer outcomes in adults than were achieved in children receiving the childhood regimen, but there were no direct comparisons between the efficacies of childhood and adult ALL regimens in adults with LBL
Summary
T-lymphoblastic lymphoma (T-LBL) represents less than 2% of adult non-Hodgkin lymphomas (NHLs), but accounts for 85% to 90% of all lymphoblastic lymphomas [1]. This high-grade lymphoma occurs mostly in males, with a high incidence of mediastinal tumors and several distinct clinical signs, including cough, shortness of breath, respiratory distress, and/or superior vena cava (SVC) syndrome [1]. Cytogenetic abnormalities show no prognostic value in adult, though they are related to an aggressive clinic course. No chromosomal or molecular abnormalities have been consistently shown to carry prognostic significance except t (9;17) (q34;3), which is associated with an aggressive clinical course in children [4, 5]
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
