A low-grade B-cell lymphoma with prolymphocytic/paraimmunoblastic proliferation and IRF4 rearrangement

Takeuchi, Kengo; Sakata, Seiji; Asaka, Reimi; Tsuyama, Naoko; Dobashi, Akito; Noguchi, Masaaki

doi:10.3324/haematol.2012.076851

Cited by 13 publications

(6 citation statements)

References 8 publications

(11 reference statements)

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“…Takeuchi et al initially reported 3 cases of low-grade BCL with IGL/IRF4 rearrangement, with an estimated frequency of 0.86% in low-grade BCL. 10 They later described one additional case (case 5). 11 To better understand this entity, we review all 5 cases and summarize the clinical and laboratory information in Table 1.…”

Section: Discussionmentioning

confidence: 99%

Primary Low-Grade B-Cell Lymphoma of Skull With Translocation Between Immunoglobulin and Interferon Regulatory Factor 4 Genes

et al. 2019

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Low-grade B-cell lymphoma with immunoglobulin ( IG) and interferon regulatory factor 4 ( IRF4) gene rearrangement is extremely rare, with only 4 cases being previously reported. In this article, we report one additional case that arises from the skull and review the literature. The patient was a 69-year-old man who presented with recurrent and disabling vertigo and was found to have a 5.0 × 1.7 cm lesion within the left posterior parietal bone. Histological examination revealed a bone lesion with diffuse lymphoid infiltrate comprising of mostly small lymphocytes with scant cytoplasm, slightly irregular nuclei and inconspicuous nucleoli, and scattered larger cells resembling prolymphocytes and paraimmunoblasts. Immunohistochemical studies showed that the neoplastic cells were positive for CD20, CD79a, PAX5, CD23, CD43, BCL-2, BCL-6, MUM-1, LEF-1, and IgM and negative for CD5, CD10, cyclinD1, SOX11, and IgD. Flow cytometric analysis identified CD5 negative and CD10 negative monoclonal B cells with lambda light chain restriction. Fluorescence in situ hybridization analysis revealed del(13q) abnormality, but was negative for IGH/BCL2, IGH/CCND1, and BIRC3/MALT1 translocations. Next-generation sequencing identified IGK-IRF4 rearrangement and BRD4 E1113 del abnormalities. Given a low clinical stage (IE) of the disease, the patient did not receive additional treatments and was free of disease at 1 year after the diagnosis.

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Section: Discussionmentioning

confidence: 99%

Primary Low-Grade B-Cell Lymphoma of Skull With Translocation Between Immunoglobulin and Interferon Regulatory Factor 4 Genes

et al. 2019

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“…A new disease entity of low-grade B-cell lymphoma has recently been proposed, which is characterized by prolymphocyte and paraimmunoblast proliferation, co-expression of MUM1 and BCL6, and reciprocal translocation of the IRF4 and immunoglobulin light chain genes. 66 Reciprocal translocation of the IRF4 and immunoglobulin genes has been reported in myeloma 67 and high-grade B-cell lymphomas, 68 but not in low-grade B-cell lymphomas. To date, 4 cases have been identified, including 3 reported cases 66 and one additional case, all of which showed no B symptoms such as fever, weight loss and night sweats.…”

Section: "Prolymphocytic/paraimmunoblastic Lymphoma" (Ppl)mentioning

confidence: 99%

“…66 Reciprocal translocation of the IRF4 and immunoglobulin genes has been reported in myeloma 67 and high-grade B-cell lymphomas, 68 but not in low-grade B-cell lymphomas. To date, 4 cases have been identified, including 3 reported cases 66 and one additional case, all of which showed no B symptoms such as fever, weight loss and night sweats. Bone marrow infiltration was 6.…”

Section: "Prolymphocytic/paraimmunoblastic Lymphoma" (Ppl)mentioning

confidence: 99%

Pathology of Indolent B-Cell Neoplasms Other than Follicular Lymphoma

Sakata

Tsuyama

Takeuchi

2014

J Clin Exp Hematopathol

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Indolent B-cell lymphomas include follicular lymphoma (FL), chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) and marginal zone lymphomas (MZLs). They are a diverse group of disorders with different clinical, morphological, immunophenotypic and genetic features. However, because of several histological similarities, such as in cell size and nodular structure, it may sometimes be difficult to differentiate them and to make a definitive diagnosis. In this review article, we summarize the histopathology of indolent B-cell neoplasms excluding FL and including hairy cell leukemia, and briefly mention recent genetic findings useful for their differential diagnosis. In addition, a provisional subtype of low-grade B-cell lymphoma, "prolymphocytic/paraimmunoblastic lymphoma", is described.

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“…Chromosomal translocations juxtaposing the IRF4 oncogene next to one of the Immunoglobulin (IG) loci has been reported also as a recurrent aberration in a subtype of mature B-cell lymphoma affecting predominantly children and young adults [105] and recently, also in 3 low-grade B-cell lymphoma cases [106].…”

Section: Irf-4mentioning

confidence: 99%

Genetic and Epigenetic Regulation of Interferon Regulatory Factor Expression: Implications in Human Malignancies

Fragale¹,

Marsili²,

Battistini³

2013

J Genet Syndr Gene Ther

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Originally identified as regulators of the type I Interferon system, the nine mammalian members of the Interferon Regulatory Factor (IRF) family are transcriptional regulators with multiple biologic functions, among which the best known are those involved in initiating and regulating many aspect of host immunity, downstream pattern recognition receptors in response to cell injuries. In addition, these versatile proteins also regulate cell differentiation, cell growth and apoptosis in several cell types, and when mutated or disregulated significantly contribute to susceptibility to and progression of several cancers. IRF-1 is the most versatile member of the family, not essential for IFN gene expression and implicated in a variety of cellular functions spanning from the development and function of various immune cells to tumor suppression activity. IRF-3, IRF-7 and IRF-9 are more specifically involved in IFN induction and antiviral responses while IRF-5 is the regulator of inflammatory cytokines expression downstream pattern recognition receptors and IRF-6 is implicated in epithelial differentiation. IRF-8 as IRF-1 is considered a tumor suppressor gene, while IRF-2 and IRF-4 have generally oncogenic activity.The present review focuses principally on the current knowledge on IRF genetic characteristics, including mutations, polymorphisms and epigenetic regulation that are implicated in oncogenesis.

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A low-grade B-cell lymphoma with prolymphocytic/paraimmunoblastic proliferation and IRF4 rearrangement

Cited by 13 publications

References 8 publications

Primary Low-Grade B-Cell Lymphoma of Skull With Translocation Between Immunoglobulin and Interferon Regulatory Factor 4 Genes

Primary Low-Grade B-Cell Lymphoma of Skull With Translocation Between Immunoglobulin and Interferon Regulatory Factor 4 Genes

Pathology of Indolent B-Cell Neoplasms Other than Follicular Lymphoma

Genetic and Epigenetic Regulation of Interferon Regulatory Factor Expression: Implications in Human Malignancies

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