Double and Triple Hit Diffuse Large B Cell Lymphomas and First Line Therapy

B cell lymphoma, unclassifiable, with features intermediate between diffuse large B cell lymphoma (DLBCL) and Burkitt lymphoma (BL) or Burkitt like lymphoma is a high grade lymphoma, with a high proliferation index and a complex karyotype mostly involving MYC and BCL-2 genes, so called “double hit” as well as BCL-6 genes, so called “triple hit”. This high grade lymphoma shows an aggressive behavior for which the most appropriate therapeutic approach is not established.

This was a single center retrospective review of patients with a confirmed diagnosis of B cell lymphoma, unclassifiable, with features intermediate between DLBCL and BL from 2006 to 2012. The definitive diagnosis in all cases was based on morphological and phenotypic features, and genetic abnormalities involving MYC, BCL-2, and BCL-6. Clinicopathological data was extracted including age, gender, primary disease location, phenotypic subtype, genetic abnormalities by FISH, IPI scores, treatment regimens and survival data. Descriptive statistical analyses were utilized. Kaplan-Meier method was used to estimate OS and log rank test was used to compare the groups. All data were analyzed using SPSS version 19.0 statistical software.

31 patients with “Double or Triple Hit” lymphomas were identified between 2006 and 2012. The age range at diagnosis was 33–87 years with median age of 71. 23 of 31 patients (74 %) were ≥ 60 years old. Male to female ratio was 1.58:1 (19:12). The median ECOG PS was 1. No patients had HIV. A majority of patients presented with extranodal disease 21 out of 31 patients and only 10 patients presented with nodal disease. The mean overall survival of the entire cohort was 28 months with ranges 3 to 40 months. 20 of 31 patients (65%) are currently alive. RCHOP was the most utilized treatment regimen in the cohort of alive patients 13 out of 20 patients (65%). Patients who presented with CNS disease had a worse prognosis when compared to the entire cohort with a mean overall survival of 13 months versus non CNS disease overall survival was 32 months (p=0.014). In patients treated with RCHOP for first line therapy the overall survival was 33 months versus 17 months for non RCHOP regimens first line (p=.048). The non RCHOP regimens included Hyper CVAD, ESHAP, and RICE.

RCHOP was the most utilized treatment regimen in the cohort of alive patients. The treatment regimen with the best efficacy in this retrospective study was RCHOP in first line treatment of “Double or Triple Hit” lymphomas. CNS disease is associated with worse prognosis in patients with “Double or Triple Hit” lymphomas in this retrospective study.

No relevant conflicts of interest to declare.