Richter's Syndrome is a highly refractory and usually fatal condition. It occurs as a result of transformation of chronic lymphocytic leukemia (CLL) or low grade lymphoma into highly aggressive lymphoma. Patients usually present with rapidly enlarging lymph nodes and systemic symptoms like night sweats, fever and weight loss. We are reporting a case of CLL presenting with similar symptoms. Initial suspicion of Richter's Syndrome proved wrong when lymph node biopsy did not reveal evidence of high grade lymphoma. Instead it showed findings consistent with cat scratch disease (CSD), later confirmed by serology. To our knowledge this is the first reported case of CSD in a patient with CLL.
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http://dx.doi.org/10.1080/10428190400004489 | DOI Listing |
Clin Hematol Int
January 2025
Service d'Hématologie Clinique et Thérapie Cellulaire Hôpital Saint-Antoine.
Individuals with chronic lymphocytic leukemia (CLL) or small lymphocytic lymphoma (SLL) have a high risk of developing other malignancies (OMs). The development of OMs may be associated with the advanced age of CLL/SLL patients, presence of a tumor-promoting microenvironment, immune alterations inherent to CLL/SLL, or chemotherapy. Importantly, the occurrence of OMs following frontline fludarabine, cyclophosphamide and rituximab (FCR) treatment is associated with a reduction in the overall survival (OS).
View Article and Find Full Text PDFEClinicalMedicine
January 2025
Monash Centre for Health Research and Implementation (MCHRI), Monash University, Clayton, VIC, Australia.
[This corrects the article DOI: 10.1016/j.eclinm.
View Article and Find Full Text PDFLeuk Lymphoma
January 2025
Centre de Recherches en Cancérologie de Toulouse, INSERM UMR1037, CNRS UMR5071, Université Toulouse III-Paul Sabatier, Toulouse, France.
In this review, we focus on the pro-oncogene MYC, the modes of deregulation in mouse and human B-cells, its undisputable importance in the evaluation of biological prognostication of patients, but also how it impacts on response to modern therapeutics, and how it should be targeted to improve the overall survival of chronic lymphocytic lymphoma (CLL) patients. After an overview of the current understanding of the molecular dysregulation of c-MYC, we will show how CLL, both in its indolent and transformed phases, has developed among other B-cell lymphomas a tight regulation of its expression through the chronic activation of B-Cell Receptors (among others). This is particularly important if one desires to understand the mechanisms at stake in the over-expression of c-MYC especially in the lymph nodes compartment.
View Article and Find Full Text PDFHematol Oncol
January 2025
Dipartimento di scienze di laboratorio ed ematologiche, Fondazione Policlinico Universitario A. Gemelli IRCCS, UOSD Leucemia Linfatica Cronica, Roma, Italy.
Hematol Rep
December 2024
Oncohematology Division, IEO European Institute of Oncology IRCCS, Via Ripamonti 435, 20141 Milan, Italy.
Richter syndrome (RS) represents a major unmet need in the lymphoma field, being refractory to chemoimmunotherapy and targeted agents. The BCL-2 inhibitor venetoclax in combination with dose-adjusted EPOCH-R chemoimmunotherapy showed promising efficacy in patients affected by RS. However, responses were not durable, suggesting the need for further treatment optimization.
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