Two decades of single-institution data reveal rare longterm survivors of relapsed/refractory Burkitt lymphoma - Letter to the editor

Q: What defines relapse timing in this study?

Early relapse is defined as relapse <6 months from first remission, while late relapse is defined as relapse ≥6 months from first remission.

Q: How are overall response rate, relapse-free survival, and overall survival defined?

Overall response rate combines complete remission and partial remission. Relapse-free survival is measured from first remission or start of first-line treatment until relapse, and overall survival is measured from first treatment failure or first relapse until death from any cause.

Q: What treatments did the long-term survivors ultimately receive?

All identified long-term survivors had previously received intensive first-line therapy and then received consolidative autologous stem cell transplant after relapse, with treatment trajectories described for the individual cases.

Sep 29, 2026

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Two decades of retrospective single-institution data identify rare long-term survivors among patients with relapsed or refractory Burkitt lymphoma (BL) and outline prognostic high-risk features. Using Memorial Sloan Kettering records from 1988 to 2021, outcomes including overall response rate, relapse-free survival, and overall survival were calculated and survival probabilities reported. The study characterizes relapse timing, treatment regimens, and subsequent salvage pathways, including consolidation with autologous stem cell transplant in the long-term survivors.

LanguageEnglish

CategoryHealthcare

FormatPDF

Pages5

Reading Time13 min

AccessFree to download

Methods and patient definitions

Study setting and endpoints

Patient baseline characteristics and treatment

High-risk features and first-line therapy

Relapse patterns and survival outcomes

Overall response and survival estimates

Long-term survivors and treatment trajectories

Case narratives and transplant details

LETTER TO THE EDITOR Two decades of single-institution data reveal rare longterm survivors of relapsed/refractory Burkitt lymphoma Burkitt lymphoma (BL) is a highly aggressive B-cell non-Hodgkin lymphoma, characterized by rapid disease progression driven by a high tumor proliferation rate 1 While intensive front-line chemoimmunotherapy has achieved 3-year overall survival (OS) rate of over 80%, a subset of patients experience refractory or relapsed (R/R) disease associated with a poor prognosis.2,3 Patients with the highest risk of relapse include those with central nervous system (CNS), marrow or peripheral blood BL, age ≥ 40, Eastern Cooperative Oncology Group score ≥ 2, and lactate dehydrogenase > 3× Upper Limit of Normal (ULN) as equally weighted independent factors.4,5 Response rates to salvage therapies vary between 30-50%, with long-term survival rates below 20% and often approaching zero. Specifically, three studies reported long-term survival in 2 of 35 patients,6 0 of 9,7 and 0 of 10,8 although one study reported 11/74 survivors.9 To evaluate our own experience and ascertain clues for long-term survival, we conducted a retrospective review of Memorial Sloan Kettering Cancer Center patients with R/R BL using a database search from January 1st, 1988, to December 21st, 2021. This study was approved by the Institutional Review Board at Memorial Sloan Kettering Cancer Center and was conducted in accordance with the Declaration of Helsinki. We identified 276 patients with BL of whom 28 (10%) received second-line therapy for relapsed (N=8) or refractory (N=20) disease. Early and late relapse were defined as relapse <6 months and ≥6 months from the time of first remission, respectively. Overall response rate (ORR) was defined as the composite of complete remission (CR) and partial remission (PR) . Relapse-free survival (RFS), and OS were calculated. RFS was defined as time from first remission or start of firstline treatment until relapse. OS was defined as time from first treatment failure or first relapse until death from any cause. Patients alive were censored at their last follow-up. Characteristics of the evaluable population are shown in Online Supplementary Table S1 . The median age was 37 years (range: 21-69 years) . High-risk baseline features include: stage IV disease (75%), leptomeningeal disease (LMD) (29%), bone marrow disease (25%) . Six patients had HIV with 3 taking antiretroviral therapy at BL diagnosis. Six patients had Epstein-Barr virus (EBV)-positive disease, defined by EBER in situ hybridization positivity on tumor specimens; 12 were EBER-negative, and 10 had unknown EBV status. The vast majority received intensive first-line therapies, e.g., CODOX/IVAC (cyclophosphamide, vincristine, doxorubicin, and high-dose methotrexate alternating with ifosfamide, etoposide, and cytarabine), DA-EPOCH (dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin), and HyperCVAD (fractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone alternating with high-dose methotrexate and cytarabine), including 20 combined with received rituximab (R) not available in the first part of the study timeframe. Responses to first-line therapy included CR (29%), PR (36%), and progression of disease (POD) (36%) . CR rates may reflect CT-defined response prior to the availability of positron emission tomography (PET). Among the 8 patients with CR, the median time to relapse was six months (95% Confidence Interval [CI]: 5-14) . Second-line regimens included R-EPOCH (N=4), R+/ -IVAC (N=6), R+/ -ICE (ifosfamide, carboplatin, and etoposide) (N=4), highdose methotrexate (MTX) +/-cytarabine (N=6), other (N=8) . A patient flow diagram shows treatment overview (Figure 1) . After second-line therapy, CR was 0%, partial response 25%, and POD 75% . The median OS for the entire cohort (measured from time of treatment failure or first relapse) was four months (95% CI: 3-8), with a 6-month OS rate of

FAQs

What defines relapse timing in this study?

Early relapse is defined as relapse <6 months from first remission, while late relapse is defined as relapse ≥6 months from first remission.

How are overall response rate, relapse-free survival, and overall survival defined?

Overall response rate combines complete remission and partial remission. Relapse-free survival is measured from first remission or start of first-line treatment until relapse, and overall survival is measured from first treatment failure or first relapse until death from any cause.

What treatments did the long-term survivors ultimately receive?

All identified long-term survivors had previously received intensive first-line therapy and then received consolidative autologous stem cell transplant after relapse, with treatment trajectories described for the individual cases.

Sep 29, 2026

Upload by Miles