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.

scholarly journals Transformation of Follicular Lymphoma to Double Hit B-Cell Lymphoma Causing Hypercalcemia in a 69-Year-Old Female: A Case Report and Review of the Literature

2014 ◽  
Vol 2014 ◽  
pp. 1-8 ◽  
Author(s):  
Sakshi Kapur ◽  
Miles B. Levin
Keyword(s):  
Follicular Lymphoma ◽  
B Cell ◽  
De Novo ◽  
Cell Lymphoma ◽  
Care Center ◽  
Tertiary Care ◽  
B Cell Lymphoma ◽  
Low Grade ◽  
Severe Hypercalcemia ◽  
Double Hit

Double hit B-cell lymphomas are rare tumors that are defined by a chromosomal breakpoint affecting the MYC/8q24 locus in combination with another recurrent breakpoint, mainly a t(14;18)(q32;q21) involving BCL2. These tumors mostly occur in adults and carry a very poor prognosis. Double hit lymphomas can occur de novo, or arise from transformation of follicular lymphoma. We report a case of a 69-year-old female with abdominal distention and progressively worsening weakness over six months. Patient presented with severe hypercalcemia and multiple intra-abdominal/pelvic masses. Histopathology results of the abdominal mass were compatible with a double hit B-cell lymphoma. However, bone marrow biopsy results showed a low grade follicular lymphoma, thus suggesting peripheral transformation of follicular lymphoma to double hit B-cell lymphoma. Patient was transferred to a tertiary care center and was started on combination chemotherapy (EPOCH: doxorubicin, etoposide, vincristine, cyclophosphamide, and prednisone). Our paper highlights not only transformation of follicular lymphoma to double hit B-cell lymphoma and the challenges encountered in diagnosing and treating these aggressive tumors, but also the association of new onset/worsening hypercalcemia in such patients.

scholarly journals “Double hit” follicular lymphoma with low proliferation index: A unique case and literature review

2017 ◽  
Vol 3 (2) ◽  
Author(s):  
Pardis Vafaii ◽  
Haipeng Shao
Keyword(s):  
Follicular Lymphoma ◽  
B Cell ◽  
Clinical Course ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Proliferation Index ◽  
Low Grade ◽  
Gene Rearrangements ◽  
Double Hit ◽  
Indolent Disease

<p>“Double hit” lymphomas (DHLs) are aggressive B-cell lymphomas with concurrent <em>c-MYC</em> and <em>BCL2</em> and/or <em>BCL6</em> gene rearrangements. DHLs are usually classified morphologically as B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma, and less commonly as DLBCL. Follicular lymphoma (FL) is characterized genetically by the presence of <em>IGH-BCL2</em> rearrangement. A subset of DHLs arises from FL by the acquisition of <em>c-MYC</em> gene rearrangement during disease progression, but FL with concurrent <em>IGH-BCL2</em> and <em>c-MYC</em> gene initial rearrangements is rarely reported. The few reported cases had different clinical courses, including some with indolent disease. We report a case of “double hit” low grade FL with both <em>c-MYC</em> and <em>BCL2</em> gene rearrangements but at low proliferation rate. Unlike the usual DHLs with aggressive clinical course, our patient showed at least partial response to intense chemotherapy. Review of the literature shows a few similar cases with variable clinical course, including a few indolent cases. These patients appear to respond better with more intense chemotherapy for DHL.</p>

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 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.

The role of translocations involving c-MYC/8q24, BCL2/18q21 and/or BCL6/3q27 genes in patients with follicular lymphoma. Retrospective analysis of single - centre data

2019 ◽  
Vol 91 (7) ◽  
pp. 52-62
Author(s):  
A E Misyurina ◽  
S K Kravchenko ◽  
A M Kovrigina ◽  
A U Magomedova ◽  
L V Plastinina ◽  
...  
Keyword(s):  
Follicular Lymphoma ◽  
B Cell ◽  
Clinical Characteristics ◽  
Cell Transformation ◽  
Cell Lymphoma ◽  
Bone Marrow Involvement ◽  
B Cell Lymphoma ◽  
High Grade ◽  
Ki 67 ◽  
Double Hit

Aim of the issue was to compare clinical characteristics and treatment results of patients with follicular lymphoma (FL) with translocations involving loci of c-MYC/8q24, BCL2/18q21 and/or BCL6/3q27 genes and patients with high - grade B-cell lymphoma [High - grade B-cell lymphoma (HGBL), double - hit (DH)]. Materials and methods. Since 2004 to 2017 years in National Research Center for Hematology 12 patients with high - grade B-cell lymphoma double - hit (HGBL DH) and 6 FL patients with translocations involving c-MYC and BCL2 and/or BCL6 had been treated. We performed a comparative analysis of clinical characterisctics in both groups. As primary endpoints was assessed frequency of complete remission (CR) or progressive disease (PD); as secondary endpoints - overall (OS) and event - free survival (EFS). Results. 5 patients with HGBL DH had c-MYC/BCL6, 7 - c-MYC/BCL2 rearrangements; 2 patients with FL had c-MYC/BCL2, 3 - c-MYC/BCL6, 1 - c-MYC/BCL2/BCL6 rearrangements. FL was represented by grade 3A in 2, grade 3B - in 4 cases, 3 of them had large - cell transformation. In HGBL DH and FL patients had no significant differences in clinical characteristics. The majority of patients had a widespread tumour, increased LDH activity, high frequency of extranodal and bone marrow involvement. Ki-67 expression level was lower in patients with FL (p

Co-existence of Follicular Lymphoma in the Lymph Node with High-grade B Cell Lymphoma in the Bone Marrow of a Patient with Spontaneous Tumor Lysis Syndrome

2020 ◽  
Vol 20 (3) ◽  
pp. 237-240
Author(s):  
Chara Giatra ◽  
Alexandros G. Sykaras ◽  
Fotis Constantinou ◽  
Victoria Gennimata ◽  
Dimitrios Sampaziotis ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Lymph Node ◽  
Follicular Lymphoma ◽  
B Cell ◽  
Cell Lymphoma ◽  
Tumor Lysis Syndrome ◽  
B Cell Lymphoma ◽  
High Grade ◽  
Tumor Lysis ◽  
Spontaneous Tumor Lysis Syndrome

A significant percentage of B-cell lymphomas are characterized by bone marrow involvement (BMI) at diagnosis. In most cases, there is a concordance between the type of lymphoma present in the lymph node and the lymphoma present in the bone marrow. Herein, we presented a sixty-seven years old female patient, who was diagnosed with High-Grade B-cell Lymphoma (HGBL) in the bone marrow, while simultaneously, in the peripheral lymph node, the presence of Follicular Lymphoma (FL) was noted. The patient was presented to the hospital with spontaneous tumor lysis syndrome, a finding compatible with the aggressive course of the HGBL. To our knowledge, this is the first case of the co-existence of HGBL in the bone marrow and FL in a lymph node, which might be attributed to merely a coincidence or to the transformation of the cells in the preferable milieu of the bone marrow.

scholarly journals Clinicopathological and genomic analysis of double-hit follicular lymphoma: comparison with high-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements

2017 ◽  
Vol 31 (2) ◽  
pp. 313-326 ◽  
Author(s):  
Masashi Miyaoka ◽  
Yara Y Kikuti ◽  
Joaquim Carreras ◽  
Haruka Ikoma ◽  
Shinichiro Hiraiwa ◽  
...  
Keyword(s):  
Follicular Lymphoma ◽  
B Cell ◽  
Cell Lymphoma ◽  
Genomic Analysis ◽  
B Cell Lymphoma ◽  
High Grade ◽  
Double Hit

scholarly journals How I treat double-hit lymphoma

Blood ◽  
2017 ◽  
Vol 130 (5) ◽  
pp. 590-596 ◽  
Author(s):  
Jonathan W. Friedberg
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
Central Nervous System Involvement ◽  
B Cell Lymphoma ◽  
World Health ◽  
Large B Cell Lymphoma ◽  
Double Hit Lymphoma ◽  
Increased Risk ◽  
Double Hit ◽  
Large B Cell

Abstract The 2016 revision of the World Health Organization (WHO) classification for lymphoma has included a new category of lymphoma, separate from diffuse large B-cell lymphoma, termed high-grade B-cell lymphoma with translocations involving myc and bcl-2 or bcl-6. These lymphomas, which occur in <10% of cases of diffuse large B-cell lymphoma, have been referred to as double-hit lymphomas (or triple-hit lymphomas if all 3 rearrangements are present). It is important to differentiate these lymphomas from the larger group of double-expressor lymphomas, which have increased expression of MYC and BCL-2 and/or BCL-6 by immunohistochemistry, by using variable cutoff percentages to define positivity. Patients with double-hit lymphomas have a poor prognosis when treated with standard chemoimmunotherapy and have increased risk of central nervous system involvement and progression. Double-hit lymphomas may arise as a consequence of the transformation of the underlying indolent lymphoma. There are no published prospective trials in double-hit lymphoma, however retrospective studies strongly suggest that aggressive induction regimens may confer a superior outcome. In this article, I review my approach to the evaluation and treatment of double-hit lymphoma, with an eye toward future clinical trials incorporating rational targeted agents into the therapeutic armamentarium.

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