Patients with Burkitt lymphoma who have central nervous system (CNS) involvement have worse prognosis, independent of first-line treatment or other factors, according to research published in Haematologica.

Investigators performed a retrospective analysis of real-world data from 641 adults diagnosed with Burkitt lymphoma between 2009 and 2018. 

The patients had a median age of 47 years, 76% were men, and 73% had stage IV disease. At diagnosis, 120 patients had CNS involvement.  The most common first-line treatment regimens were:

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  • CODOX-M/IVAC (cyclophosphamide, doxorubicin, vincristine, and high-dose methotrexate, alternating with ifosfamide, etoposide, and cytarabine), given to 30% of patients
  • HyperCVAD/MA (cyclophosphamide, vincristine, doxorubicin, and dexamethasone, alternating with high-dose methotrexate and cytarabine), given to 30% of patients
  • DA-EPOCH-R (dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, and rituximab), given to 28% of patients.

In a multivariate model, CNS involvement was independently associated with HIV infection (odds ratio [OR], 1.84; P =.017), poor performance status (OR, 2.13; P =.004), involvement of 2 or more extranodal sites (OR, 2.94; P <.001), and bone marrow involvement (OR, 2.80; P <.001).

First-line treatment regimens did not differ significantly according to CNS involvement at baseline (P =.93). However, the complete response rate was significantly lower in patients with CNS disease than in those without it — 59% and 77%, respectively (OR, 0.45; P <.001). 

The estimated 3-year progression-free survival (PFS) rate was 46% for patients with CNS disease and 69% for those without it (hazard ratio [HR], 2.02; 95% CI, 1.52-2.67; P <.001).  The median PFS was 1.1 years and not reached, respectively.

The estimated 3-year overall survival (OS) rate was 49% for patients with CNS disease and 74% for those without it (HR, 2.18; 95% CI, 1.61-2.94; P <.001). The median OS was 2.6 years and not reached, respectively.

After adjustment for characteristics associated with poor outcomes, CNS involvement remained an independent predictor of PFS (HR, 1.53; 95% CI, 1.14-2.06; P =.004) and OS (HR, 1.62; 95% CI, 1.18-2.22, P =.003).

The 3-year risk of CNS recurrence was significantly lower among patients who received CODOX-M/IVAC (4%) or hyperCVAD/MA (3%) than among those who received DA-EPOCH-R (13%; adjusted sub-distribution HR, 3.57; 95% CI, 1.83-6.97; P <.001).

Recurrences were more likely to involve the CNS after DA-EPOCH-R (40%) than after the other 2 two regimens (16%, P <0.001). According to the study authors, this finding may be explained by suboptimal adherence to CNS staging and intrathecal therapy among patients who received DA-EPOCH-R.

“Our results have important implications for the management of BL [Burkitt lymphoma] in clinical practice, given the rarity of the disease and the paucity of randomized trials,” the authors wrote. “Further prospective studies are needed to optimize realistic delivery of CNS-directed prophylaxis with all standard regimens and to mitigate the incidence of CNS recurrence.”

Disclosures: Some study authors declared affiliations with biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of authors’ disclosures.


Zayac AS, Evens AM, Danilov A, et al. Outcomes of Burkitt lymphoma with central nervous system involvement: Evidence from a large multicenter cohort study. Haematologica. 2021;106(7):1932-1942. doi:10.3324/haematol.2020.270876