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http://dx.doi.org/10.3324/haematol.2017.168930 | DOI Listing |
Int J Mol Sci
January 2025
Immunology Division, Department of Internal Medicine and Hematology, Semmelweis University, 1088 Budapest, Hungary.
Schnitzler syndrome is a unique autoinflammatory disease, of which 747 cases have been described worldwide to date. The main features of the syndrome are a triad of recurrent urticaria, monoclonal IgM gammopathy, systemic inflammation associated with recurrent fever, joint and bone pain, and atypical bone remodeling (osteosclerosis). The abnormal activation of the NLRP3 inflammasome produces IL-1, which drives the disease pathology, but it also involves IL-6 and IL-18.
View Article and Find Full Text PDFMicromachines (Basel)
January 2025
Center of Competence at Mechatronics and Clean Technologies-MIRACle, "Acad. G. Bontchev" Str. 4, 1113 Sofia, Bulgaria.
Chronic lymphocytic leukemia (CLL), the most common type of leukemia, remains incurable with conventional therapy. Despite advances in therapies targeting Bruton's tyrosine kinase and anti-apoptotic protein BCL-2, little is known about their effect on red blood cell (RBC) aggregation in blood flow. In this study, we applied a microfluidic device and a newly developed Software Image Flow Analysis to assess the extent of RBC aggregation in CLL patients and to elucidate the hemorheological effects of the commonly applied therapeutics Obinutuzumab/Venetoclax and Ibrutinib.
View Article and Find Full Text PDFCancers (Basel)
January 2025
Division of Hematology and Medical Oncology, University of Washington, Seattle, WA 98195, USA.
Pathway inhibitors targeting Bruton tyrosine kinase (BTK) and B-cell lymphoma-2 (BCL-2) have dramatically changed the treatment landscape for both treatment-naïve and relapsed/refractory chronic lymphocytic leukemia (CLL). However, with increased utilization, a growing number of patients will experience progressive disease on both agents. This subgroup of "double refractory" patients has limited treatment options and poor prognosis.
View Article and Find Full Text PDFBlood Adv
January 2025
Fred Hutchinson Cancer Research Center, Seattle, Washington, United States.
Some CLL patients who develop progressive disease (PD) during treatment with covalent Bruton tyrosine kinase inhibitors (cBTKi) acquire pathway resistance mutations in BTK or PLCG2. Here, we report gene mutation data from paired baseline and PD peripheral blood samples from 52 patients (zanubrutinib, n=24; ibrutinib, n=28) who, at an early median follow-up time of 25.7 months, progressed on zanubrutinib or ibrutinib treatment in the ALPINE trial (NCT03734016).
View Article and Find Full Text PDFNMC Case Rep J
December 2024
Department of Neurosurgery, Graduate School of Biomedical and Health Sciences, Hiroshima University, Hiroshima, Hiroshima, Japan.
Older patients represent a unique and vulnerable subgroup, requiring careful consideration when determining treatment options. Treatment-related leukoencephalopathy is commonly observed in older patients months to years after receiving radiotherapy and/or methotrexate for primary central nervous system lymphoma (PCNSL). Tirabrutinib is an orally available, highly selective, and potent second-generation Bruton's tyrosine kinase inhibitor (BTKi) approved for treating recurrent/refractory PCNSL in Japan.
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