<scp>B</scp>‐cell lymphoma, unclassifiable, with features intermediate between diffuse large <scp>B</scp>‐cell lymphoma and burkitt lymphoma: study of 39 cases

Perry, Anamarija M.; Crockett, David G.; Davé, Bhavana J.; Althof, Pamela A.; Winkler, L; Smith, Lynette M.; Aoun, Patricia; Chan, Wing C.; Fu, Kai; Greiner, Timothy C.; Bierman, Philip J.; Bociek, R. Gregory; Vose, Julie M.; Armitage, Jamés O.; Weisenburger, Dennis D.

doi:10.1111/bjh.12343

Cited by 73 publications

(68 citation statements)

References 31 publications

(61 reference statements)

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“…FISH testing is indicated in patients in whom BL is a consideration and in those who have a .20% incidence of MYC translocations, such as HGBL-NOS, plasmablastic lymphoma, and patients with histologic transformation from antecedent FL. 18 Immunohistochemistry can be useful for screening patients who require FISH because it is inexpensive, accessible, and routinely used in clinical laboratories. 19 The proliferation marker Ki-67 is always increased in BL but is more variable in HGBL-DH and is not a reliable marker for screening patients that require FISH.…”

Section: Clinical Presentationmentioning

confidence: 99%

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Approach to the diagnosis and treatment of high-grade B-cell lymphomas with MYC and BCL2 and/or BCL6 rearrangements

Sesques

Johnson

2017

Blood

137

116

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High-grade B-cell lymphomas (HGBLs) with MYC and BCL2 and/or BCL6 rearrangements, so-called “double-hit” lymphomas (HGBL-DH), are aggressive lymphomas that form a separate provisional entity in the 2016 revised World Health Organization Classification of Lymphoid Tumors. Fluorescence in situ hybridization (FISH) will be required to identify HGBL-DH and will reclassify a subset of diffuse large B-cell lymphomas (DLBCLs) and HGBLs with features intermediate between DLBCL and Burkitt lymphoma into this new category. Identifying patients with HGBL-DH is important because it may change clinical management. This poses a challenge for centers that may not be ready to handle the additional workload and financial burden associated with the increase in requests for FISH testing. Herein, we review the mechanisms of deregulation of these oncogenes. We identify the factors associated with a poor prognosis and those that can guide diagnostic testing. Restricting FISH analysis to the 10% of DLBCL patients who have a germinal center B-cell phenotype and coexpress MYC and BCL2 proteins would be cost-effective and would identify the subset of patients who are at highest risk of experiencing a relapse following conventional therapy. These patients may benefit from intensified chemotherapy regimens or, ideally, should enroll in clinical trials investigating novel regimens.

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Section: Clinical Presentationmentioning

confidence: 99%

“…10,18 BCL2 is located on the chromosome arm 18q21 and was originally described in follicular lymphoma (FL). 30 BCL2 is a bona fide oncogene, 31 but lymphocytes overexpressing BCL2 require additional genomic alterations before developing overt lymphoma.…”

Section: Introductionmentioning

confidence: 99%

Approach to the diagnosis and treatment of high-grade B-cell lymphomas with MYC and BCL2 and/or BCL6 rearrangements

Sesques

Johnson

2017

Blood

137

116

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“…Perry et al [17] reported 39 cases of BCLu-DLBCL/BL over a period of 15 years. In his study majority of patients presented with advanced-stage disease (62 %) with median overall survival (OS) of only 9 months and the 5-year OS was 30 %.…”

Section: Discussionmentioning

confidence: 99%

Clinicopathological Analysis of B Cell Lymphomas, Unclassifiable; with Features Intermediate Between Diffuse Large B-Cell Lymphoma and Burkitt Lymphoma in a Tertiary Care Hospital in Southern India

Selvi

Kar

Basu

et al. 2015

Indian J Hematol Blood Transfus

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(2008) to overcome the problems of difficulty in classifying certain lymphomas having overlapping morphological, immunophenotypical and genetic features. To study the clinicopathological profile of BCLu-DLBCL/BL. Crosssectional study over 3 year period in the Haematology section of Department of Pathology in a large teaching hospital in Southern India from January 2011 to December 2013. All the cases reported as BCLu-DLBCL/BL were collected and the clinical, morphological and immunohistochemical parameters were analyzed. Descriptive statistics. There were seven cases, four males and three females, of age ranging from 20 to 70 years. Five cases had extranodal involvement. Four cases had Burkitt morphology with strong Bcl2 positivity and absent CD10 expression. One case had the morphology and immunophenotype that of typical BL, along with strong positivity to Bcl2 suggesting a double hit hypothesis. Two cases had morphology and immunophenotype of BL with low Ki 67. Three patients on follow up had adverse outcome. BCLu-DLBCL/BL, a provisional category in WHO 2008 is useful in classifying the cases not meeting the criteria for classical BL or DLBCL. Each of these cases was interesting with different sites of involvement, different morphological features and immunophenotype with most of the patients on follow up ending with a grave prognosis.

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Section: Casementioning

confidence: 99%

“…However, we continue to recommend CNS prophylaxis for patients with HGBL-NOS based on the increased risk observed in BCL-U and Burkitt lymphoma; in retrospective series, many patients with BCL-U morphology presented with IPI 3 to 5 even in the absence of MYC translocations. 53,54 However, this issue clearly warrants further study in larger datasets with central pathology review.Outcome. We favor dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, and rituximab (DA-EPOCH-R) for fit patients with HGBL-DH based on retrospective 26,52,55 and prospective data.…”

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confidence: 99%

How I treat patients with aggressive lymphoma at high risk of CNS relapse

Chin

Cheah

2017

Blood

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Central nervous system (CNS) relapses are an uncommon yet devastating complication of non-Hodgkin lymphomas. The identification of patients at high risk of secondary CNS relapse is therefore paramount. Retrospective data indicate prophylactic CNS-directed therapies may reduce the risk of CNS involvement; however, no consensus exists about dose, timing, or route of therapy. In addition, prophylaxis is not without risk of treatment-related complications and morbidity. Here, we present a series of case vignettes highlighting our approach to common dilemmas encountered in routine clinical practice. We review the method of assessing CNS relapse risk, factors that increase the likelihood of relapse including histologic subtype, MYC rearrangement, protein expression, and extranodal involvement, and review our clinical practice based on available evidence in administering CNS-directed prophylaxis. (Blood. 2017;130(7):867-874) IntroductionNon-Hodgkin lymphomas are biologically and clinically diverse hematological malignancies. Treatment is influenced by patient fitness, disease biology, and tumor burden. Identifying patients at high risk of central nervous system (CNS) relapse is important as the outcome of secondary CNS lymphoma is poor. [1][2][3][4] Risk of CNS progression is influenced by histologic subtype and subtype-specific clinicopathologic features (eg, site of involvement, protein expression, or gene rearrangements). Patients with highly aggressive lymphomas (eg, lymphoblastic, Burkitt lymphoma) are at high risk and frontline protocols include CNS-directed prophylaxis. In contrast, indolent lymphomas rarely involve the CNS and CNS prophylaxis is not required. Between these extremes fall diffuse large B-cell lymphoma (DLBCL), high-grade B-cell lymphoma (HGBL) with MYC and BCL2 or BCL6 rearrangements, so-called "double-hit" lymphomas (HGBL-DH), and (nodal) peripheral T-cell lymphomas (PTCLs). The addition of rituximab has slightly reduced CNS relapse in DLBCL, probably through superior systemic control as there is negligible CNS penetration of the drug across the intact blood-brain barrier. 1,5 However, ;4% of unselected patients with DLBCL treated with prednisone, vincristine, cyclophosphamide, doxorubicin, and rituximab (R-CHOP) experience CNS relapse, 6,7 and considerable efforts have been made to identify those at greatest risk. In this review, we will summarize our approach to this problem with case vignettes drawn from our practice. How I identify patients at increased risk for CNS relapseMany investigators have examined risk factors for CNS relapse, but studies have yielded inconsistent results, due to heterogeneity in patient populations, treatment, and/or limited sample size. [8][9][10][11][12][13][14][15][16] To address some of these limitations, Schmitz et al developed a 6-point score from 2164 patients treated on prospective German studies and validated in 1597 patients with DLBCL treated with R-CHOP in the British Columbia Cancer Agency (BCCA) Lymphoid Malignancies Database. 6 The components of the s...

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B‐cell lymphoma, unclassifiable, with features intermediate between diffuse large B‐cell lymphoma and burkitt lymphoma: study of 39 cases

Cited by 73 publications

References 31 publications

Approach to the diagnosis and treatment of high-grade B-cell lymphomas with MYC and BCL2 and/or BCL6 rearrangements

Approach to the diagnosis and treatment of high-grade B-cell lymphomas with MYC and BCL2 and/or BCL6 rearrangements

Clinicopathological Analysis of B Cell Lymphomas, Unclassifiable; with Features Intermediate Between Diffuse Large B-Cell Lymphoma and Burkitt Lymphoma in a Tertiary Care Hospital in Southern India

How I treat patients with aggressive lymphoma at high risk of CNS relapse

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