Purpose Of Review: We review the genetic foundations of different rare lymphomas to examine their shared origins. These data indicate the potential application of genomics to improve the diagnosis and treatment of these rare diseases.
Recent Findings: Next generation sequencing technologies have provided an important window into the genetic underpinnings of lymphomas. A growing body of evidence indicates that although some genetic alterations are specific to certain diseases, others are shared across different lymphomas. Many such genetic events have already demonstrated clinical utility, such as BRAF V600E that confers sensitivity to vemurafenib in patients with hairy cell leukemia.
Summary: The rareness of many lymphoma subtypes makes the conduct of clinical trials and recruitment of significant numbers of patients impractical. However, a knowledge of the shared genetic origins of these rare lymphomas has the potential to inform 'basket' clinical trials in which multiple lymphoma subtypes are included. These trials would include patients based on the presence of alterations in targetable driver genes. Such approaches would be greatly strengthened by a systematic assessment of significant patient numbers from each subtype using next generation sequencing.
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http://dx.doi.org/10.1097/MOH.0000000000000435 | DOI Listing |
Clin Genitourin Cancer
January 2025
Department of Pathology, The First Medical Centre, Chinese PLA General Hospital, Beijing, China. Electronic address:
Objective: To investigate the clinical characteristics, pathology, imaging features, and prognosis of primary renal lymphoma (PRL), a rare malignancy.
Patients And Method: We conducted a retrospective review of 14 PRL cases diagnosed between January 2009 and January 2022, with follow-up data collected from medical records.
Results: The study included 14 patients (7 males, 7 females), with a mean age of 60.
Cancer Med
February 2025
ERN-EuroBloodNet, Hôpital St Louis/Université Paris 7, Paris, France.
Introduction: Burkitt lymphoma (BL) is a rare and aggressive subtype of non-Hodgkin's lymphoma. Several studies have identified prognostic factors (PFs) for disease progression and mortality among adults with BL. However, there is no consensus on risk stratification based on PFs.
View Article and Find Full Text PDFSwiss Med Wkly
January 2025
Department of Internal Medicine, Clinic for Medical Oncology and Hematology, Municipal Hospital Zurich Triemli, Zurich, Switzerland.
Introduction: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare disease, with unique diagnostic challenges and often dismal outcome. There are no widely accepted treatment guidelines available. Lymphoma-like regimens with or without autologous or allogenic transplantation were the cornerstone of most therapeutic concepts.
View Article and Find Full Text PDFIntroduction: Angioimmunoblastic T-cell lymphoma (AITL) is a rare and aggressive lymphoma with a poor prognosis. AITL is associated with Epstein-Barr virus (EBV)-positive B cells in most cases, suggesting a possible role for the virus in the pathobiology of AITL. Cell lines from AITL patients do not exist and models of human AITL are needed.
View Article and Find Full Text PDFRadiol Case Rep
March 2025
Loyola University Medical Center and Loyola University Chicago, 2160 S First Ave, Maywood, IL 60153, USA.
Klippel-Feil syndrome (KFS) is a rare congenital disorder characterized by the fusion of cervical vertebrae, with a clinical presentation that can vary widely due to genetic and phenotypic diversity. While KFS can occur as an isolated anomaly, it is often associated with other congenital conditions, such as Sprengel deformity, which may present with or without an omovertebral bone, complicating diagnosis and management. This particular case also involves diffuse large B-cell lymphoma (DLBCL), the most common subtype of non-Hodgkin lymphoma.
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