Drug therapy for double-hit lymphoma

Richter transformation (RT) is defined as the transformation of chronic lymphocytic leukemia (CLL) to a high-grade B cell lymphoma. It usually carries a dismal prognosis and represents an unmet need for novel therapeutic interventions. We report a case of an 80-year-old male who developed double-hit (DH) RT with translocations of MYC and BCL6 after 5 years of watchful waiting for standard-risk CLL. He was treated with induction therapy consisting of 4 cycles of anthracycline-based chemoimmunotherapy (CIT) and 2 cycles of platinum-based CIT with intrathecal methotrexate for CNS prophylaxis followed by continuous maintenance therapy with ibrutinib. He achieved complete remission after the induction therapy. At the time of writing, four and a half years after the diagnosis with DH-RT, he remains in complete remission without evidence of RT or CLL. The novel therapeutic approach used in successful treatment of this patient should be further explored.

show abstract

“…REPOCH has been used as the standard CIT for DH/TH lymphomas. 9 He responded well-achieving complete remission following anthracycline-based CIT.…”

Section: Discussionmentioning

confidence: 95%

Successful Non-Transplant Treatment of Double Hit Richter Transformation with Long-Term Remission

Seegobin

Moustafa

Jiang

et al. 2021

BLCTT

View full text Add to dashboard Cite

show abstract

“…However, our data suggested that the R-DAEPOCH regimen may not overcome haematological toxicity, and treatment for prevention of neutropenia and infection should be provided. The prognosis and outcomes of HGBCL when treated with R-CHOP are worse than those when treated with other regimens, with 5-year PFS and OS rates of approximately 20-30% [19]. Therefore, higher-intensity chemotherapy regimens, such as R-DAEPOCH, R-Hyper-CVAD/MA (rituximab, cyclophosphamide, vincristine, doxorubicin, dexamethasone/methotrexate and cytarabine) and R-CODOX-M/IVAC (rituximab, cyclophosphamide, vincristine, doxorubicin, methotrexate, ifosfamide, etoposide, and cytarabine), have been used for HGBCL patients, although standard of treatment is not established.…”

Section: Discussionmentioning

confidence: 99%

A multicentre retrospective study of primary breast diffuse large B-cell and high-grade B-cell lymphoma treatment strategies and survival

et al. 2020

View full text Add to dashboard Cite

Objectives:: Primary breast diffuse large B-cell lymphoma (PB-DLBCL) and primary breast highgrade B-cell lymphoma (PB-HGBCL) are rare extranodal aggressive B-cell lymphomas with distinct characteristics. Reliable data regarding appropriate treatment of these specific entities are lacking due to their rarity. Methods:: We reviewed 36 patients diagnosed at four Chinese medical centres between January 2008 and December 2018. Data regarding clinicopathological features, therapeutic evaluation and central nervous system (CNS) relapse were collected, and overall survival (OS) and progression-free survival (PFS) were calculated. Results:: Among the 36 patients, there were 29 PB-DLBCL patients and 7 PB-HGBCL patients. The 5-year OS for PB-DLBCL and PB-HGBCL was 75.9% and 28.6%, respectively. The 5-year PFS for PB-DLBCL and PB-HGBCL was 69.0% and 14.3%, respectively. The R-DAEPOCH regimen was significantly more effective in PB-DLBCL patients than the R-CHOP regimen (5year OS: 78.9% vs 62.5%, P=0.024; 5-year PFS: 73.7% vs 50.0%, P=0.037) but resulted in more severe myelosuppression (P=0.025). The rate of CNS relapse was 17.2% in PB-DLBCL patients and 28.6% in PB-HGBCL patients; the difference was not significant (P=0.602). The R-DAEPOCH regimen did not predominantly reduce CNS recurrence as expected (P=0.616). The Cox proportional hazards model revealed that risk stratification and triple expression were independent prognostic factors. Conclusion:: Current treatments, including more intensive chemotherapy regimens, achieve good control of the disease. Novel drugs combined with cellular immunotherapy initially show promising therapeutic effects, and more clinical trials are required to confirm these effects further.

show abstract

“…At present, no standard treatment protocol has been developed for this disease. 16 Clinical trials are still underway to determine which may confer better progression free survival and overall survival.…”

Section: Discussionmentioning

confidence: 99%

Disseminated Double-Hit Lymphoma in a Young Adult

Damian¹,

Cancio²

2021

Phil Journal Path

View full text Add to dashboard Cite

High-grade B-cell lymphoma with MYC and BCL2 and-or BCL6 rearrangements, also called doublehit lymphoma, is an aggressive mature B-cell lymphoma that carries both MYC and BCL2 and/or BCL6 translocations. This can present as a diagnostic challenge since clinical characteristics, morphology and immunophenotype are not accurate indicators of underlying genetic aberrations in this type of lymphoma. We report a case of a 25-year-old male who presented with a one-year history of cough, gradually increasing abdominal girth and jaundice. Definitive diagnosis was made post-mortem with additional ancillary studies using immunohistochemistry staining and fluorescent in-situ hybridization studies.

show abstract

Drug therapy for double-hit lymphoma

Cited by 12 publications

References 76 publications

Successful Non-Transplant Treatment of Double Hit Richter Transformation with Long-Term Remission

Successful Non-Transplant Treatment of Double Hit Richter Transformation with Long-Term Remission

A multicentre retrospective study of primary breast diffuse large B-cell and high-grade B-cell lymphoma treatment strategies and survival

Disseminated Double-Hit Lymphoma in a Young Adult

Contact Info

Product

Resources

About