Burkitt

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Despite excellent results in frontline therapy, particularly in pediatric age, refractory Burkitt lymphoma still remains a therapeutic challenge, with dismal outcome. The prognosis is very poor, ranging from less than 10% to 30-40%, with longer survival only in transplanted patients. On account of the paucity of data, mostly reporting on small series of patients, with heterogeneous characteristics and salvage treatments, at present it is impossible to draw definitive conclusions on the treatment of choice for this difficult to treat subset of patients...

BACKGROUND: Solid organ transplantation and bone marrow/hematologic stem cell transplantation recipients face a heightened risk of developing malignancies or cancer as a result of immunosuppression. Posttransplant lymphoproliferative disorders (PTLD) are a range of disorders from benign lymphoid growth to lymphoma found post-transplant. Risk factors for PTLD include high immunosuppressive use and oncogenic effects of Epstein-Barr virus (EBV). There is a lack of comprehensive clinical and pathological documentation of PTLD cases among Saudi patients, and the available data are limited to a few case reports...

Natural killer (NK) cells' unique ability to kill transformed cells expressing stress ligands or lacking major histocompatibility complexes (MHC) has prompted their development for immunotherapy. However, NK cells have demonstrated only moderate responses against cancer in clinical trials and likely require advanced genome engineering to reach their full potential as a cancer therapeutic. Multiplex genome editing with CRISPR/Cas9 base editors (BE) has been used to enhance T cell function and has already entered clinical trials but has not been reported in human NK cells...

INTRODUCTION: Patients with systemic lupus erythematosus (SLE) are at a higher risk of developing cancer, particularly hematological malignancies such as lymphoma and leukemia. However, existing studies on this topic that assess cancer incidence following SLE diagnosis are limited. In addition, SLE can be diagnosed after cancer, although such cases in children have been rarely reported. CASE REPORT: We present the case of a 2.6-year-old boy who presented to our institute with fever and abdominal pain...

BACKGROUND: Multi-factorial reasons are an induction to cause cancer. Different infections and infestations with viruses, bacteria, and parasites have been detected for many years to be related to human carcinogenesis. PURPOSE: The study aimed to review all ideas of tumor carcinogenesis and its associations with parasitic infections and infestations. METHODS: We reviewed several articles (published and imprinted) by selecting, extracting, and synthesizing data about the relationship between cancers and parasites...

Many types of malignancies have been associated with immunodeficiency states, especially patients who are HIV positive. Burkitt lymphoma (BL) is one of those malignancies associated with HIV and it presents in three varieties. The endemic form is primarily seen in children, and it is associated with the Epstein-Barr virus (EBV). In this form, patients with Burkitt's present with a large jaw mass. The second variety is seen in older adults. These patients usually present with abdominal and pelvic masses. This subtype is more prominent in the United States...

BACKGROUND: 16p11.2 proximal deletion and duplication syndromes (Break points 4-5) (593KB, Chr16; 29.6-30.2mb - HG38) are observed to have highly varied phenotypes, with a known propensity for lifelong psychiatric problems. This study aimed to contribute to a research gap by qualitatively exploring the challenges families with 16p11.2 deletion and duplication face by answering three research questions: (1) What are parents' perceptions of the ongoing support needs of families with children who have 16p11...

We present 2 diagnostically challenging cases of pediatric/adolescent relapsed/refractory aggressive mature B-cell non-Hodgkin lymphoma (B-NHL) within the spectrum of Burkitt lymphoma and diffuse large B-cell lymphoma and illustrate the different therapeutic regimens that are employed for pediatric and adult cancer centers. Both cases displayed varying-sized lymphoma cells with occasional single prominent nucleoli and heterogeneous BCL2 expression. Cytogenetics revealed complex karyotypes with t(8:14)(q24.2;q32) and IGH::MYC rearrangement by FISH...

INTRODUCTION: The role of age and sex in the presentation and outcome of endemic Burkitt lymphoma (BL) has not been studied recently. This study analysed these factors in 934 patients with BL who had received cyclophosphamide and intrathecal methotrexate as treatment. METHODS: Records of 934 children diagnosed with BL from 2004 to 2015 were obtained from our Paediatric Oncology Networked Database (POND) cancer registry. Age at diagnosis, sex, disease stage, time to diagnosis, delay in diagnosis, completion of treatment, rate of abandonment, and one-year survival rates were recorded and statistically analysed...

Non-Hodgkin's Lymphoma (NHL) in people living with Human Immunodeficiency Virus/Acquired Immunodeficiency Syndrome  ( HIV/AIDS) still constitutes a reality of high morbidity and mortality, sometimes not respecting the patient's degree of immunosuppression as it can even occur in those with a high CD4+ T lymphocyte count. Burkitt's lymphoma, in this sense, has been shown to be one of the main subtypes of this condition in this group of patients. This case report concerns a 32-year-old man diagnosed with metastatic gastric Burkitt's lymphoma after one month of his admission to a tertiary hospital for neurological complaints...

Epstein-Barr virus (EBV) contributes to the development of a significant subset of human lymphomas. As a herpes virus, EBV can transition between a lytic state which is required to establish infection and a latent state where a limited number of viral antigens are expressed which allows infected cells to escape immune surveillance. Three broad latency programs have been described which are defined by the expression of viral proteins RNA, with latency I being the most restrictive expressing only EBV nuclear antigen 1 (EBNA1) and EBV-encoded small RNAs (EBERs) and latency III expressing the full panel of latent viral genes including the latent membrane proteins 1 and 2 (LMP1/2), and EBNA 2, 3, and leader protein (LP) which induce a robust T-cell response...

SMARCA4 encodes one of two mutually exclusive ATPase subunits in the BRG/BRM associated factor (BAF) complex that is recruited by transcription factors (TFs) to drive chromatin accessibility and transcriptional activation. SMARCA4 is among the most recurrently mutated genes in human cancer, including ∼30% of germinal center (GC)-derived Burkitt lymphomas. In mice, GC-specific Smarca4 haploinsufficiency cooperated with MYC over-expression to drive lymphomagenesis. Furthermore, monoallelic Smarca4 deletion drove GC hyperplasia with centroblast polarization via significantly increased rates of centrocyte recycling to the dark zone...

Cryptosporidium species cause watery diarrhea in several vertebrate hosts, including humans. Most apparently, immunocompetent-infected individuals remain asymptomatic, whereas immunocompromised may develop severe or chronic cryptosporidiosis. We report here the case of a 6-year-old girl undergoing chemotherapy for Burkitt lymphoma who experienced multiple episodes of watery diarrhea during her hospital stay. Microscopic examination of her stool sample revealed oocysts of Cryptosporidium species. The rapid immunochromatographic test was also positive for Cryptosporidium species...

Epstein-Barr virus (EBV) is a lymphotropic virus that causes diseases ranging from a flu-like illness called infectious mononucleosis to nasopharyngeal carcinoma, Burkitt's lymphoma, and central nervous system (CNS) infection. Detection of EBV DNA is usually done using whole blood samples taken from the patients. We undertook the detection of EBV in blood, cerebrospinal fluid (CSF), and saliva by real-time quantitative PCR in two patients, one with a history of nasopharyngeal carcinoma, and the other having a case of viral encephalitis...

No abstract text is available yet for this article.

Burkitt lymphoma is a highly aggressive and rapidly growing B cell non-Hodgkin lymphoma with heterogeneous pattern of manifestations. We present a rare case of a 39-year-old immunocompetent male with double localization of Burkitt lymphoma: gastroduodenal and ileal tract. The gastric location is extremely rare, and the association with another site is unusual with only few reports in the literature. Imaging modalities play a crucial role in correct diagnosis as it can manifest as a focal mass or as segmental wall thickening...

Lymphomas are a heterogeneous group of diseases that originate from T, B or natural killer cells. Lymphoma treatment is based on chemotherapy, radiotherapy, and monoclonal antibody (mAb) or other immunotherapies. The P-selectin glycoprotein ligand 1 (PSGL-1) is expressed at the surface of hematological malignant cells and has been shown to have a pro-oncogenic role in multiple myeloma and lymphoma. Here, we investigated the expression and therapeutic potential of PSGL-1 in T and B cell lymphomas. By flow cytometry analysis, we found that PSGL-1 was expressed in both T and B cell-derived lymphoma cell lines but generally at higher levels in T cell lymphoma cell lines...

Dimeric naphthopyranones are known to be biologically active, however, for the corresponding monomeric naphthopyranones this information is still elusive. Here the first enantioselective total synthesis of semi-viriditoxic acid as well as the synthesis of semi-viriditoxin and derivatives is reported. The key intermediate in the synthesis of naphthopyranones is an α,β-unsaturated δ-lactone, which we synthesized in two different ways (Ghosez-cyclization and Grubbs ring-closing metathesis), while the domino-Michael-Dieckmann reaction of the α,β-unsaturated δ-lactone with an orsellinic acid derivative is the key reaction...

OBJECTIVES: Dominant-activating (DA) lesions in RAC2 have been reported in 18 individuals to date. Some have required haematopoietic stem cell transplantation (HSCT) for their (severe) combined immunodeficiency syndrome phenotype. We aimed to investigate clinical and cellular features of a kindred harbouring a novel variant in RAC2 p.Ile21Ser (I21S) to better understand DA lesions' phenotypic spectrum. METHODS: Clinical and immunological information was collated for seven living individuals from the same kindred with RAC2 p...