Adult Burkitt leukemia and lymphoma

Blum, Kristie A.; Lozanski, Gerard; Byrd, John C.

doi:10.1182/blood-2004-02-0405

Cited by 500 publications

(477 citation statements)

References 77 publications

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“…The genetic hallmark of all three subtypes is the re-arrangement of the C-MYC oncogene that contributes to lymphomagenesis through dysregulation of cell cycle, cellular differentiation, apoptosis, cellular adhesion, and metabolism [5]. Histologically the tumor cells are round and uniform with multiple nucleoli and basophilic lipid-vacuole laden cytoplasm.…”

Section: Discussionmentioning

confidence: 99%

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Multiple cranial nerve palsies in immunodeficiency subtype of Burkitt lymphoma

Ali

Kalla

2018

Journal of Community Hospital Internal Medicine Perspectives

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Burkitt lymphoma is a late complication of HIV, and bilateral cranial nerve palsies are extremely rare in patients with AIDS. A twenty year old Caucasian male with known congenital HIV who had been non-adherent with anti-retroviral therapy presented with multiple cranial nerve palsies and was eventually diagnosed with Burkitt lymphoma. Before chemotherapy, he was started on radiation therapy to the brain, meninges, and base of skull with the intent of improving cranial nerve palsies and preventing further neurological sequelae since the cranial nerve palsies were dense and there was concern that intrathecal chemotherapy would have less penetration than radiation. He eventually died due to overall disease burden. We hereby present what we believe is the first reported case of Burkitt lymphoma presenting with bilateral facial, vestibulocochlear, left abducens, and mandibular nerve palsies. Recognition of different presentations of Burkitt lymphoma is extremely important as it would aid in early diagnosis and initiation of both chemotherapy and anti-retroviral therapy potentially leading to improved outcomes.

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Section: Discussionmentioning

confidence: 99%

“…Histologically the tumor cells are round and uniform with multiple nucleoli and basophilic lipid-vacuole laden cytoplasm. Cells have a proliferation index of > 99% on Ki67 staining and express CD10, CD19, CD20, CD22, CD79a and surface IgM [5]. …”

Section: Discussionmentioning

confidence: 99%

Multiple cranial nerve palsies in immunodeficiency subtype of Burkitt lymphoma

Ali

Kalla

2018

Journal of Community Hospital Internal Medicine Perspectives

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“…Direct extension from anterior mediastinal or axillary nodal disease is the most common type of spread to the chest wall, but occasionally, a chest wall mass due to lymphoma may arise from skin or soft tissue in the absence of mediastinal or axillary nodal or other extranodal involvement [2,3]. Sporadic BL typically presents as an intraabdominal process and may involve the kidney, pancreas, liver, spleen, or ovary [4]. This paper is a case of primary skin or soft tissue involvement of the chest wall in the absence of apparent nodal mass or visceral disease as the initial manifestation of BL.…”

Section: Discussionmentioning

confidence: 99%

Primary Burkitt Lymphoma of the Chest Wall

Hess

2010

Clinical Lymphoma Myeloma and Leukemia

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Burkitt lymphoma (BL) originating in the skin and soft tissue at any site is exceedingly rare. This paper is about a case of primary sporadic BL that presented as an isolated, rapidly enlarging chest wall mass arising from skin and/or soft tissue in an adult. As with other BL presentations, this patient was treated with aggressive chemotherapy with central nervous system (CNS) chemoprophylaxis, but he later died because of sepsis.

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“…Among IgH cis-regulatory elements, Em was expected to be the critical c-myc deregulator in lymphomagenesis. However, Em-c-myc transgenic mice expressed c-myc in B-cell progenitors and, thus, developed an immature form of lymphoma [25] that differed from human Burkitt lymphoma tumors that harbor a mature B-cell signature [24]. In Burkitt lymphoma, c-myc translocation breakpoints occur either in the V(D)J (endemic Burkitt lymphoma) or in switch regions (sporadic Burkitt lymphoma), both of which keep the 3 0 RR intact.…”

Section: C-myc 3 0 Rr and Lymphomagenesismentioning

confidence: 99%

“…The cyclin D1, cyclin D3 or c-maf translocations often observed in myelomas are obviously linked to CSR [23]. However c-myc translocation, a typical hallmark of Burkitt lymphoma, is related to either SHM or CSR [24]. Among IgH cis-regulatory elements, Em was expected to be the critical c-myc deregulator in lymphomagenesis.…”

Section: C-myc 3 0 Rr and Lymphomagenesismentioning

confidence: 99%

The IgH 3′ regulatory region and its implication in lymphomagenesis

et al. 2010

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The 3 0 regulatory region (3 0 RR) located downstream of the IgH gene is the master element that controls class switch recombination and sustains high-level transcription at the plasma-cell stage. This latter role suggests that the 3 0 RR may be involved in oncogene deregulation during the frequent IgH translocation events associated with B-cell malignancies. A convincing demonstration of the essential contribution of 3 0 RR in lymphomagenesis has been provided by transgenic animal models. The mouse 3 0 RR shares a strong structural homology with the regulatory regions located downstream of each human Ca gene. Mouse models exploring the role of the 3 0 RR in B-cell physiology and in malignancies should provide useful indications about the pathophysiology of human cell lymphocyte proliferation.Key words: B cells . Cellular proliferation . Oncology IntroductionDuring precursor B-cell differentiation, genes encoding heavy (H) and light chains of an Ig molecule are somatically assembled from germline DNA. This process, named V(D)J recombination, occurs in the bone marrow prior to antigenic challenge. In germinal centers during the antigen-dependent stages, variable (V) regions become the target of somatic hypermutation (SHM) in activated B cells allowing the generation of high-affinity Ig. In mature B cells, class switch recombination (CSR) deletes the constant (C) m region and replaces it with a downstream C H gene. This enables B cells to express various Ig isotypes but still retain antigen specificity. Once activated, B cells differentiate into Ig-secreting plasma cells. During B-cell development all these events (V(D)J recombination, SHM, CSR, Ig synthesis) are coupled with transcriptional accessibility of the IgH loci. IgH transcription is controlled by the functional interactions of multiple promoters, enhancers and insulators spread among the 2.5 megabases of the locus. Among them, the upstream Em enhancer and the 3 0 regulatory region (3 0 RR) stand out as major players. Chromosomal translocations linking oncogenes to these elements are often implicated as the cause of B-cell malignancies. In this brief review of relevant knock-out and transgenic mouse models, we underline how the physiological functions of the IgH 3 0 RR might translate into a critical role in oncogene deregulation during lymphomagenesis.From the El cis-activating element to the 3 0 RR Thirty years ago, Em was the first transcriptional enhancer discovered upstream of the m gene (Fig. 1A) [1][2][3]. Em deletion in mice confirmed its role in controlling access to the locus prior to D-J recombination, but, moreover, showed its dispensability for CSR and SHM [4]. Eventually, several more transcriptional enhancers were identified at the 3 0 end of the locus. hs1,2 was identified 12.5-kb downstream of the mouse Ca (Fig. 1A) 3306Mini-Review differentiation stages, while hs4 is active during the pre-B-cell stage and throughout B-cell development (Fig. 1A) [8,9]. The modest activity of each of the 3 0 RR elements, however, contributes to a synergic...

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Adult Burkitt leukemia and lymphoma

Cited by 500 publications

References 77 publications

Multiple cranial nerve palsies in immunodeficiency subtype of Burkitt lymphoma

Multiple cranial nerve palsies in immunodeficiency subtype of Burkitt lymphoma

Primary Burkitt Lymphoma of the Chest Wall

The IgH 3′ regulatory region and its implication in lymphomagenesis

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