Burkitt lymphoma (BL) is common in sub-Saharan Africa (SSA). In high-income countries, BL is highly curable with chemotherapy. However, there are few prospective studies from SSA describing nonpediatric BL and no regional standard of care. Thirty-five participants age 15 years or older with newly diagnosed BL were enrolled in Malawi from 2013 to 2018. Chemotherapy was administered according to institutional guidelines, with concurrent antiretroviral therapy if HIV infected. Median age was 21 years (range, 15-61) and 15 participants (43%) were HIV infected. Twenty-seven participants (77%) had stage III to IV disease, and 19 (54%) had Eastern Cooperative Oncology Group performance status >1. Among HIV-infected participants, median CD4 count was 130 (range, 29-605) and 10 (67%) had suppressed HIV viral load. Four participants (11%) died before receiving chemotherapy. First-line chemotherapy consisted of: cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) (n = 22 [71%]); infusional etoposide, prednisolone, vincristine, cyclophosphamide, and doxorubicin (n = 4 [13%]); high-dose methotrexate-based chemotherapy (n = 4 [13%]); and rituximab plus CHOP (n = 1 [3%]). Among 28 evaluable participants, 14 (50%) achieved a complete response. Median overall survival (OS) was 7 months; 1-year OS was 40% (95% confidence interval [CI], 24%-56%). Sixteen (73%) of 22 deaths were a result of disease progression. Compared with CHOP, more intensive chemotherapy was associated with decreased mortality (hazard ratio, 0.24; 95% CI, 0.05-1.02; = .05). This is among the best characterized prospective cohorts of nonpediatric BL in SSA. Most deaths resulted from progressive BL. Patients who received more intensive therapy seemed to have better outcomes. Defining optimal approaches is an urgent priority in SSA.
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http://dx.doi.org/10.1182/bloodadvances.2018029199 | DOI Listing |
Transl Pediatr
December 2024
Department of Hematology, Chongqing Medical University Affiliated Children's Hospital, Chongqing, China.
Background: Post-transplant lymphoproliferative disease (PTLD) is a significant complication that can arise following solid organ transplantation or hematopoietic stem cell transplantation. It encompasses a spectrum of lymphoproliferative lesions, ranging from benign reactive hyperplasia to malignant tumors, and is among the most severe complications following liver transplantation in children. It is essential for clinicians to gain a comprehensive understanding of the prevention, clinical manifestations, early diagnosis, and treatment strategies for PTLD in order to reduce mortality rates.
View Article and Find Full Text PDFJ Med Virol
January 2025
The Wistar Institute, Philadelphia, Pennsylvania, USA.
Epstein-Barr virus (EBV) is a ubiquitous human ɣ-herpesvirus implicated in various malignancies, including Burkitt's lymphoma and gastric carcinomas. In most EBV-associated cancers, the viral genome is maintained as an extrachromosomal episome by the EBV nuclear antigen-1 (EBNA1). EBNA1 is considered to be a highly stable protein that interacts with the ubiquitin-specific protease 7 (USP7).
View Article and Find Full Text PDFJ Nat Med
January 2025
Chongqing Academy of Chinese Materia Medica, Chongqing University of Chinese Medicine, Chongqing, 402760, China.
Non-Hodgkin lymphomas (NHL), including diffuse large B-cell lymphoma (DLBCL), Burkitt lymphoma (BL), and follicular lymphoma (FL), predominantly arise from B cells undergoing germinal center (GC) reactions. The transcriptional repressor B-cell lymphoma 6 (BCL6) is indispensable for GC formation and contributes to lymphomagenesis via its BTB domain-mediated suppression of target genes. Dysregulation of BCL6 underpins the pathogenesis of GC-derived NHL.
View Article and Find Full Text PDFAdv Anat Pathol
January 2025
Institut d'Investigacions Biomediques August Pi i Sunyer (IDIBAPS).
Aggressive B-cell lymphomas are a biologically and clinically very heterogeneous group of tumors that may be related to different stages of B-cell differentiation development. This review aims to summarize recent advances in the understanding of these tumors with a focus on the practical approach to the diagnosis of these entities. We analyze the defining characteristics of the different subtypes of aggressive B-cell lymphomas, including nodal and extranodal diffuse large B-cell lymphoma, virus-associated lymphomas, terminally differentiated B-cell lymphomas, high-grade B-cell lymphomas, and Burkitt lymphoma.
View Article and Find Full Text PDFAm J Ophthalmol Case Rep
March 2025
Department of Ophthalmology, Hospital Sultan Idris Shah, Serdang, Malaysia.
Purpose: To highlight a case report of high-grade primary lacrimal sac Burkitt lymphoma in a young adult.
Observation: A 25-year-old gentleman was referred to the oculoplastic center for left eye medial canthal progressive swelling at the level below the medial canthal tendon for two months associated with tearing. He was initially treated for preseptal cellulitis but failed to respond to antibiotics.
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