<i><scp>MYC</scp>/<scp>BCL</scp>6</i> double‐hit lymphoma (<scp>DHL</scp>): a tumour associated with an aggressive clinical course and poor prognosis

Li, Shaoying; Desai, Parth; Lin, Pei; Yin, C. Cameron; Tang, Guilin; Wang, Xuan J.; Konoplev, Sergej; Khoury, Joseph D.; Bueso‐Ramos, Carlos E.; Medeiros, L. Jeffrey

doi:10.1111/his.12884

Cited by 86 publications

(70 citation statements)

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“…We detected double expression of MYC and BCL-2 in 27% of our R-CHOP treated DLBCL patients, which corroborates the findings reported in previous studies, both regarding the frequency of cases with dual expression and the inferior survival evidenced in these cases [12,20,22,25]. More recently, DHL with rearrangements concerning MYC and BCL-6 have been associated with a worse prognosis [16,17], however, limited data regarding the impact of DHE of MYC and BCL-6 on survival has been published [17], presumably due to the rarity of cases expressing MYC and BCL-6. The rarity of this combination is indeed reflected in our cohort with only one patient found to overexpress MYC and BCL-6 in the absence of an overexpression of BCL-2.…”

Section: Discussionsupporting

confidence: 91%

“…Consequently, the published data on MYC/BCL-6 DHL is limited with only single case reports and a few case series available [16][17][18]. An even rarer entity is the so-called triplehit lymphoma (THL) within which the recurrent cytogenetic abnormalities involve chromosomal rearrangements of the MYC, BCL-2 and BCL-6 genes has been reported; however, the exact incidence of THL is unknown and the World Health Organization (WHO) has no specific classification for this subset of patients [19].…”

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confidence: 99%

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A population-based study of cellular markers in R-CHOP treated diffuse large B-cell lymphoma patients

et al. 2016

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Section: Discussionsupporting

confidence: 91%

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confidence: 99%

A population-based study of cellular markers in R-CHOP treated diffuse large B-cell lymphoma patients

et al. 2016

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“…The cutoffs for positivity for CD10, BCL6, and MUM1 were 30% as were used by Hans and colleagues [24]. The positive cutoffs for MYC and BCL2 were ≥ 40% and ≥ 50% of cells, respectively, as have been used earlier and by others [32, 33, 36–39]. …”

Section: Methodsmentioning

confidence: 99%

Prognostic impact of history of follicular lymphoma, induction regimen and stem cell transplant in patients withMYC/BCL2double hit lymphoma

Saksena

Desai

et al. 2016

Oncotarget

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MYC/BCL2 double hit lymphoma (DHL) has been the subject of many studies; however, no study has systemically compared the clinicopathologic features and prognostic factors between patients with de novo disease versus those with a history of follicular lymphoma (FL). In addition, the prognostic importance of several other issues remains controversial in these patients. In this retrospective study, we assess 157 patients with MYC/BCL2 DHL including 108 patients with de novo disease and 49 patients with a history of FL or rarely other types of low-grade B-cell lymphoma. Patients received induction chemotherapy regimens including 61 R-CHOP, 31 R-EPOCH, 29 R-Hyper-CVAD, and 23 other regimens. Thirty-nine patients received a stem cell transplant (SCT) including 31 autologous and 8 allogeneic. Sixty-two patients achieved complete remission (CR) after induction chemotherapy. Median overall survival (OS) was 19 months. Clinicopathologic features were similar between patients with de novo tumors versus those with a history of FL (P > 0.05). Using multivariate analysis, achieving CR, undergoing SCT, stage and the International Prognostic Index were independent prognostic factors for OS. Stem cell transplantion was associated with improved OS in patients who failed to achieve CR, but not in patients who achieved CR after induction chemotherapy. In conclusion, patients with MYC/BCL2 DHL who present with de novo disease and patients with a history of FL have a similarly poor prognosis. Achievement of CR, regardless of the induction chemotherapy regimen used, is the most important independent prognostic factor. Patients who do not achieve CR after induction chemotherapy may benefit from SCT.

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“…[2][3][4][5][6][7] Within the population of patients with DHL, a worse prognosis has been associated with multiple clinicopathologic features present at diagnosis, including B-cell lymphoma unclassifiable (BCLU) histologic classification, 2 evidence of MYC-IG translocation, 2,8,9 bone marrow involvement, 2,4 elevated serum lactate dehydrogenase, 4,6 and CNS involvement. 4,6 With the recognition of the poor survival experienced by patients with DHL, chemotherapy intensification has been explored as one strategy for improving outcomes in this population.…”

Section: Introductionmentioning

confidence: 99%

Outcomes of Patients With Double-Hit Lymphoma Who Achieve First Complete Remission

Landsburg

Falkiewicz

Maly

et al. 2017

JCO

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Patients with double-hit lymphoma (DHL) rarely achieve long-term survival following disease relapse. Some patients with DHL undergo consolidative autologous stem-cell transplantation (autoSCT) to reduce the risk of relapse, although the benefit of this treatment strategy is unclear. MethodsPatients with DHL who achieved first complete remission following completion of front-line therapy with either rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) or intensive front-line therapy, and deemed fit for autoSCT, were included. A landmark analysis was performed, with time zero defined as 3 months after completion of front-line therapy. Patients who experienced relapse before or who were not followed until that time were excluded. ResultsRelapse-free survival (RFS) and overall survival (OS) rates at 3 years were 80% and 87%, respectively, for all patients (n = 159). Three-year RFS and OS rates did not differ significantly for autoSCT (n = 62) versus non-autoSCT patients (n = 97), but 3-year RFS was inferior in patients who received R-CHOP compared with intensive therapy (56% v 88%; P = .002). Three-year RFS and OS did not differ significantly for patients in the R-CHOP or intensive therapy cohorts when analyzed by receipt of autoSCT. The median OS following relapse was 8.6 months. ConclusionIn the largest reported series, to our knowledge, of patients with DHL to achieve first complete remission, consolidative autoSCT was not associated with improved 3-year RFS or OS. In addition, patients treated with R-CHOP experienced inferior 3-year RFS compared with those who received intensive front-line therapy. When considered in conjunction with reports of patients with newly diagnosed DHL, which demonstrate lower rates of disease response to R-CHOP compared with intensive front-line therapy, our findings further support the use of intensive front-line therapy for this patient population.

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MYC/BCL6 double‐hit lymphoma (DHL): a tumour associated with an aggressive clinical course and poor prognosis

Abstract: MYC/BCL6 DHL is an aggressive B cell lymphoma and patients often have an aggressive clinical course and poor prognosis, similar to patients with MYC/BCL2 DHL.

Cited by 86 publications

References 35 publications

A population-based study of cellular markers in R-CHOP treated diffuse large B-cell lymphoma patients

A population-based study of cellular markers in R-CHOP treated diffuse large B-cell lymphoma patients

Prognostic impact of history of follicular lymphoma, induction regimen and stem cell transplant in patients withMYC/BCL2double hit lymphoma

Outcomes of Patients With Double-Hit Lymphoma Who Achieve First Complete Remission

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