scholarly journals Double-hit lymphoma: optimizing therapy

Hematology ◽  
2021 ◽  
Vol 2021 (1) ◽  
pp. 157-163
Author(s):  
Kieron Dunleavy
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
High Grade ◽  
Multicenter Studies ◽  
Not Otherwise Specified ◽  
Double Hit Lymphoma ◽  
Genomic Studies ◽  
Novel Approaches ◽  
Poor Outcomes

Abstract Aggressive B-cell lymphoma is a heterogeneous entity with disparate outcomes based on clinical and pathological characteristics. While most tumors in this category are diffuse large B-cell lymphoma (DLBCL), the recognition that some cases have high-grade morphology and frequently harbor MYC and BCL2 and/or BCL6 translocations has led to their separate categorization. These cases are now considered distinct from DLBCL and are named “high-grade B-cell lymphoma” (HGBL). Most are characterized by distinct rearrangements, but others have high-grade morphological features without these and are called HGBL-not otherwise specified. Studies have demonstrated that this group of diseases leads to poor outcomes following standard rituximab, cyclophosphamide, hydroxydaunorubicin, vincristine, and prednisone therapy; retrospective and recent single-arm, multicenter studies suggest they should be approached with dose-intense treatment platforms. As yet, this has not been validated in randomized trial settings due to the rarity of these diseases. In the relapsed and refractory setting, novel approaches such as anti-CD19 chimeric antigen receptor T cells and antibodies against CD19 have demonstrated high efficacy in this subgroup. Recently, genomic studies have made much progress in investigating some of the molecular underpinnings that drive their lymphomagenesis and have paved the way for testing additional novel approaches.

scholarly journals Tumor necrosis factor- and interleukin-6-producing high-grade B-cell lymphoma, not otherwise specified in the pleura

2018 ◽  
Vol 10 ◽  
pp. 1-3 ◽  
Author(s):  
Shoko Nakayama ◽  
Mitsuhiro Matsuda ◽  
Tatsuya Adachi ◽  
Sanae Sueda ◽  
Kayo Ueda ◽  
...  
Keyword(s):  
Tumor Necrosis Factor ◽  
B Cell ◽  
Interleukin 6 ◽  
Tumor Necrosis ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
High Grade ◽  
Not Otherwise Specified ◽  
Necrosis Factor

Does CNS Prophylaxis Prevent CNS Relapse in Patients with High-Grade B-Cell Lymphoma Double-Hit and not Otherwise Specified?

2019 ◽  
Vol 19 ◽  
pp. S254-S255
Author(s):  
Anna Misyurina ◽  
Sergey Kravchenko ◽  
Aminat Magomedova ◽  
Yana Mangasarova ◽  
Elena Baryakh ◽  
...  
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
High Grade ◽  
Cns Prophylaxis ◽  
Not Otherwise Specified ◽  
Cns Relapse ◽  
Double Hit

Challenges and Opportunities for High-grade B-Cell Lymphoma With MYC and BCL2 and/or BCL6 Rearrangement (Double-hit Lymphoma)

2019 ◽  
Vol 42 (3) ◽  
pp. 304-316 ◽  
Author(s):  
Dongfeng Zeng ◽  
Aakash Desai ◽  
Fangfang Yan ◽  
Tiejun Gong ◽  
Haige Ye ◽  
...  
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
High Grade ◽  
Double Hit Lymphoma ◽  
Challenges And Opportunities ◽  
Double Hit

scholarly journals High-Grade B-Cell Lymphoma Not Otherwise Specified (HGBL, NOS) in the Maxillary Sinus Mimicking Periapical Inflammation: Case Report and Review of the Literature

2021 ◽  
Vol 11 (19) ◽  
pp. 8803
Author(s):  
Andrea Brody ◽  
Csaba Dobo-Nagy ◽  
Karoly Mensch ◽  
Zsuzsanna Oltyan ◽  
Judit Csomor ◽  
...  
Keyword(s):  
B Cell ◽  
Maxillary Sinus ◽  
Poor Prognosis ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
High Grade ◽  
B Cell Lymphomas ◽  
Not Otherwise Specified ◽  
Systemic Spread ◽  
Periapical Inflammation

High-grade B-cell lymphoma not otherwise specified is listed as a new group in the WHO 2017 statement as a subtype of aggressive, mature B-cell lymphomas with a poor prognosis. To our knowledge, no description of this genetic type of maxillary lymphoma has appeared in the literature until now; thus, our case provides valuable data on its symptoms, clinical behavior, response to treatment and survival rate. The present report describes the early diagnosis and treatment of an extremely rare histological subtype of B-cell lymphoma, a case of high-grade B-cell lymphoma not otherwise specified, localized in the maxillary sinus and mimicking signs and symptoms of periapical inflammation. After chemotherapy, the presented patient showed complete remission without relapse and systemic spread. As far as we know, this is the first reported case of this rare type of lymphoma associated with the maxillary sinus. Considering that high-grade B-cell lymphomas are aggressive tumors with rapid growth and poor prognosis, which are often misdiagnosed in the early stages as inflammatory disease, it is relevant to highlight the importance of a detailed evaluation of clinical signs and radiological findings during diagnosis, especially if they contradict each other.

scholarly journals The Effect of Therapy on High Grade B Cell Lymphoma, Not Otherwise Specified and Outcomes in Comparison with Double Hit Lymphoma

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 4224-4224 ◽  
Author(s):  
Jonathan Rush ◽  
Alison R. Sehgal ◽  
Christine G. Roth ◽  
Michael Boyiadzis
Keyword(s):  
Overall Survival ◽  
B Cell ◽  
Standard Therapy ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
High Grade ◽  
University Of Pittsburgh ◽  
Double Hit Lymphoma ◽  
Double Hit ◽  
The University

Abstract Background: High grade B cell lymphoma (HGBL) is a heterogeneous entity with morphologic and genetic features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma (BL). Many patients with HGBL also have concurrent MYC, BCL2 and/or BCL6 rearrangements documented by FISH, the so-called double-hit lymphoma (DHL), which has now been defined as a separate entity in the updated WHO classification in 2016 as HGBL with rearrangements of MYC and BCL2 and/or BCL6. Other HGBL without MYC and BCL2 or BCL6 have been termed HGBL, NOS. HGBL, NOS and DHL are considered clinically aggressive. The best therapeutic approach for HGBL, NOS is unclear, and outcomes of patients with DHL in comparison to HGBL, NOS are not well established. Objective: The aim of this study was to evaluate and describe the outcomes and practice patterns for patients with HGBL, NOS. Additionally, we compared the survival of patients with HGBL, NOS to those with DHL. Materials and Methods: This retrospective cohort study was conducted at the University of Pittsburgh Medical Center. All patients identified as HGBL by pathology review at the University of Pittsburgh Hematopathology Department from 2010-2014 were included. Outcomes between standard therapy with R-CHOP and high intensity therapies with either R-EPOCH or R-HyperCVAD were compared for those patients with HGBL. Survival curves were generated with the Kaplan-Meier method, and overall survival was compared using the log-rank test. Cox-regression analysis was used to adjust for covariates. Results: 50 patients with newly diagnosed HGBL without a double-hit genotype were identified. Of these patients, 38 received R-CHOP (63%), R-EPOCH (21%) or R-HyperCVAD (16%). Baseline characteristics between treatment groups (Table 1) revealed an older age (p=0.033) and more frequent germinal center genotype (p=0.023) in those treated with R-CHOP. Median follow-up for this group was 18 months. There was no difference in the overall survival (OS) between R-CHOP and higher intensity regimens (p=0.540) (Figure 1). Median survival was not reached in either group. 2-year survival was 76%. Only a high-risk IPI score retained prognostic significance with OS in multivariate analysis (p=0.022). This compares favorable to 13 cases of DHL treated with R-CHOP, R-EPOCH, or R-HyperCVAD during a similar time period for whom the median overall survival was 8.9 months (p=0.016) Conclusion: Both standard therapy with R-CHOP and higher intensity therapies appear to be effective in the treatment of HGBL without double-hit genotype. IPI score remains prognostically significant. Outcomes in patients with HGBL, NOS appear favorable in comparison with patients with DHL. Figure Figure. Disclosures No relevant conflicts of interest to declare.

scholarly journals High-grade B-cell Lymphoma with MYC and BCL2 Rearrangement Arising from Follicular Lymphoma: Presentation as a Large Peripancreatic Mass

Diagnostics ◽  
2020 ◽  
Vol 10 (3) ◽  
pp. 157
Author(s):  
Anna Shestakova ◽  
Sherif Rezk ◽  
Dara Ghasemizadeh ◽  
Ali Nael ◽  
Xiaohui Zhao
Keyword(s):  
Lymph Node ◽  
Follicular Lymphoma ◽  
B Cell ◽  
Cell Lymphoma ◽  
Inguinal Lymph Node ◽  
B Cell Lymphoma ◽  
Low Grade ◽  
High Grade ◽  
Double Hit Lymphoma ◽  
Double Hit

Follicular lymphoma, the second most common non-Hodgkin lymphoma (NHL), primarily affects adults and shows an indolent clinical course. Rare cases of follicular lymphoma transform to a high-grade B-cell lymphoma with MYC and BCL2 rearrangements or “double-hit lymphoma”. Transformation to a “double-hit lymphoma” portends a worse prognosis and requires aggressive treatment. We report a comprehensive clinical, pathologic and radiographic review of a patient with previously undiagnosed low-grade follicular lymphoma that transformed into a “double-hit lymphoma”. The patient presented with a large heterogeneous mass 16 x 19 cm involving pancreatic head and neck and a mildly enlarged inguinal lymph node. Positron emission tomography (PET) study demonstrated Fluorodeoxyglucose (18F) (FDG)-avid peripancreatic mass. Tissue biopsy demonstrated a high-grade B-cell lymphoma with rearrangements t(14;18) and MYC, leading to the diagnosis of high-grade B-cell lymphoma with MYC and BCL2 rearrangements. Excisional biopsy of an inguinal lymph node demonstrated low-grade follicular lymphoma. Clonality studies demonstrated the same immunoglobulin clone V7-4 in inguinal lymph node and peripancreatic mass. Therefore, diagnosis of a high-grade B-cell lymphoma with MYC and BCL2 rearrangements that transformed from a low-grade follicular lymphoma was rendered. It is ultimately important to establish a tissue-based diagnosis at the different sites that are involved with lymphoma. Patient proceeded with the aggressive treatment with dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin and rituximab (EPOCH-R) treatment.

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