Translocation (3;8)(q27;q24) in two cases of triple hit lymphoma

Motlló, Cristina; Grau, Javier; Juncà, Jordi; Ruiz, Neus; Mate, José-Luís; Orna, Elisa; Navarro, José-Tomás; Vives, Susana; Sancho, Juan‐Manuel; Esteban, Daniel; Granada, Isabel; Feliú, Evarist; Ribera, Josep‐Marǐa; Millá, Fuensanta

doi:10.1016/j.cancergencyto.2010.08.018

Cited by 20 publications

(16 citation statements)

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“…13 Lymphomas with MYC, BCL2, and BCL6 rearrangement/translocations, so-called triple-hit lymphoma (THL) are very rare, and only case reports and a few small case series have been published. [14][15][16][17][18][19][20][21][22][23][24][25][26][27] Although one would hypothesize and initial reports suggest that patients with these tumors would be similar to patients with DHL, the available literature is inadequate to support this hypothesis, and it is unknown whether THLs are more like MYC/BCL2 DHLs or MYC/BCL6 DHLs. In addition, the immunophenotype of THL cases is not well characterized.…”

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confidence: 99%

Triple-hit B-cell Lymphoma With MYC, BCL2, and BCL6 Translocations/Rearrangements

Wang

et al. 2015

American Journal of Surgical Pathology

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Lymphomas with translocations/rearrangements of MYC, BCL2, and BCL6, so-called triple-hit B-cell lymphoma, are rare, and few studies on these tumors are available in the literature. We report 11 cases of triple-hit B-cell lymphoma and characterize their clinicopathologic findings. All patients were men, with a median age of 64 years (range, 45 to 80 y), and 4 patients had antecedent or concurrent follicular lymphoma. Using the 2008 World Health Organization classification, these cases were classified as: 5 B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma; 4 DLBCL; 1 DLBCL with concurrent follicular lymphoma; and 1 low-grade follicular lymphoma. All cases were positive for CD10, BCL2, and FOXP1. Ten of 11 cases were positive for CD20. MYC expression was high in 10/11 (91%), BCL6 was positive in 8/11 (73%), and MUM1/IRF4 was positive in 6/11 (55%) cases. T-cell antigens, TdT, and Epstein-Barr virus-encoded RNA were negative in all cases. Ten of 11 cases showed a high proliferation index-70% to 100%, and the follicular lymphoma had a 30% proliferation rate. Using most algorithms, all cases belonged to germinal center B-cell-like group. All patients received standard or more aggressive immunochemotherapy regimens. Three patients had no response to chemotherapy; 4 patients showed a partial response; 2 patients had complete remission after chemotherapy; and 2 patients had just begun chemotherapy. Three patients underwent a stem cell transplant. The median follow-up time was 5.3 months. Five patients died, and 6 patients were alive at last follow-up. Two patients who underwent stem cell transplant after complete response to chemotherapy were in remission with 16 to 19 months of clinical follow-up. In summary, triple-hit lymphomas are clinically aggressive tumors associated with a poor prognosis. Patients often respond poorly to chemotherapy, but a subset may completely respond to chemotherapy followed by stem cell transplant.

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confidence: 99%

Triple-hit B-cell Lymphoma With MYC, BCL2, and BCL6 Translocations/Rearrangements

Wang

et al. 2015

American Journal of Surgical Pathology

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“…In consequence, it is important to select the right therapeutic strategy as they vary accordingly. Standard chemotherapy used for DLBCL or BL is not efficient in this case, mostly because of the particular genetic profile and the worse clinical course 22 . Some studies have outlined a median survival time 5 months shorter than for the more frequent lymphomas, classical DLBCL or BL.…”

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confidence: 99%

Case report. A rare case of triple-hit diffuse large B-cell lymphoma of the parotid gland in a patient with Sjogren’s syndrome

Birceanu

Evsei

Dumitru

et al. 2017

Romanian Journal of Rhinology

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A rare case of triple-hit diffuse large B-cell lymphoma of the parotid gland in a patient with Sjogren's syndrome CASE REPORT INTRODUCTIONPrimary lymphoma of the salivary gland is rare, accounting for 2-5% of all salivary neoplasms. Diffuse large B-cell lymphomas (DLBCL) are uncommon, most salivary gland lymphoid malignancies being lowgrade lymphomas with MALT (mucosa associated lymphoid tissue) lymphomas being the most common. The majority of primary salivary gland DLBCLs appear to arise from MALT type lymphomas in a background of sialadenitis associated with autoimmune disorders, such as Sjogren syndrome [1][2][3][4] . The pathophysiology of Non-Hodgkin Lymphoma (NHL) is unknown. However, some risk factors are related to NHL development, such as acquired or druginduced immunodeficiency, some autoimmune diseases or Sjogren's syndrome 5 . Double and triple-hit B-cell lymphoma (DHL, THL) are rare lymphoma subtypes usually associated with poor prognosis. These specific subtypes are defined by two or three recurrent chromosome translocations: MYC/8q24loci, usually in combination with the t(14;18)(q32; q21) bcl-2 gene and/or BCL6 3q27 chromosomal translocation. Here, we present one case with diffuse large B-cell lymphoma associated with follicular lymphoma transformed to THL 6 . ABSTRACT BACKGROUND. Primary malignant lymphomas of the salivary gland are rare, accounting for 2% of salivary gland tumors and 5% of all extranodal lymphomas. The clinical presentation is not particularly characteristic, a feature that usually leads to diagnostic and treatment delays. CASE REPORT.We report a case of a parotid gland triple-hit diffuse large B-cell (DLBCL) lymphoma associated with follicular lymphoma in a 76-year-old female patient with a unique personal history, which included a diagnosis of Sjogren Syndrome and exposure to a toxic working environment with pesticides. Diffuse large B-cell lymphomas are uncommon given the fact that most lymphoid malignancies are low-grade lymphomas, with MALT (mucosa associated lymphoid tissue) lymphomas being the most common. Triple-hit DLBCL are extremely rare and the diagnosis can be challenging. Parotidectomy, as the first step, must be followed by histopathology and immunohistochemistry for final diagnosis and treatment.CONCLUSION. This case highlights the fact that B-cell lymphoma in the salivary gland can be unrecognized due to unspecific symptoms and requires immunohistochemistry studies for confirmation. It is important to recognize triple-hit lymphoma due to its worse prognosis and differentiated treatment. Patients with Sjogren syndrome have additional risk factors for progression to lymphoma.

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“…Mutations of the BCL-6 gene can lead to tumor progression secondary to the repression of the p53 signal [8] . Triple-hit lymphomas are also well known for having a tumultuous disease course given their spread to extranodal sites on a more frequent and rapid basis [9] . Triple-hit lymphomas have a poor prognosis as compared to other types of lymphoma, with an average survival time of four months [9] .…”

Section: Discussionmentioning

confidence: 99%

“…Triple-hit lymphomas are also well known for having a tumultuous disease course given their spread to extranodal sites on a more frequent and rapid basis [9] . Triple-hit lymphomas have a poor prognosis as compared to other types of lymphoma, with an average survival time of four months [9] . Standard chemotherapy is often ineffective for the treatment of triple-hit lymphomas.…”

Section: Discussionmentioning

confidence: 99%

“Triple-triple” hit lymphoma

Mushtaq

Singhi

Stuart

2015

CRIM

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Triple-hit lymphoma is a rare but serious form of Non-Hodgkin's lymphoma that is known to have a worse prognosis than diffuse large B-cell lymphoma (DLBCL) or Burkitt lymphoma (BL) alone, with a survival time of only a few months. A triple-hit lymphoma has cytogenetic abnormalities consistent with chromosomal rearrangements of the BCL-6, BCL-2, and c-MYC genes, with MYC translocations having a worse prognosis. This specific type of lymphoma is medically challenging to treat, as standard chemotherapy is often ineffective. We report a case of a 47-year-old African American male with triple-hit lymphoma as a result of a series of progressive malignant transformations over a time course of three years.

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Translocation (3;8)(q27;q24) in two cases of triple hit lymphoma

Cited by 20 publications

References 12 publications

Triple-hit B-cell Lymphoma With MYC, BCL2, and BCL6 Translocations/Rearrangements

Triple-hit B-cell Lymphoma With MYC, BCL2, and BCL6 Translocations/Rearrangements

Case report. A rare case of triple-hit diffuse large B-cell lymphoma of the parotid gland in a patient with Sjogren’s syndrome

“Triple-triple” hit lymphoma

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