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| http://dx.doi.org/10.1136/ard.44.10.723-b | DOI Listing |
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Rituximab intervention in management of thrombocytopenia in patients with systemic lupus erythematosus: A single centre experience.
Lupus
February 2025
Division of Rheumatology, Department of Internal Medicine, Istanbul University-Cerrahpasa, Istanbul, Turkey.
Objective: Assessment of the efficacy and safety of rituximab (RTX) in treating thrombocytopenia of systemic lupus erythematosus (SLE) patients unresponsive to conventional treatments, highlighting its potential as an alternative to the expensive intravenous immunoglobulin (IVIG), and investigating the optimal number of RTX cycles for complete remission (CR).
Methods: A retrospective analysis was conducted on RTX utilization between 2016 and 2023 at our center. Medical records of SLE patients with thrombocytopenia, resistant to conventional treatments, were reviewed.
Functional validation of a novel STAT3 'variant of unknown significance' identifies a new case of STAT3 GOF syndrome and reveals broad immune cell defects.
Clin Exp Immunol
January 2025
Garvan Institute of Medical Research, Darlinghurst, NSW, Australia.
Introduction: Signal transducer and activator of transcription 3 (STAT3) orchestrates crucial immune responses through its pleiotropic functions as a transcription factor. Patients with germline monoallelic dominant negative or hypermorphic STAT3 variants, who present with immunodeficiency and/or immune dysregulation, have revealed the importance of balanced STAT3 signaling in lymphocyte differentiation and function, and immune homeostasis. Here, we report a novel missense variant of unknown significance in the DNA-binding domain of STAT3 in a patient who experienced hypogammaglobulinemia, lymphadenopathy, hepatosplenomegaly, immune thrombocytopenia, eczema, and enteropathy over a 35-year period.
View Article and Find Full Text PDFPrimary versus Secondary Immune Thrombocytopenia (ITP): A Meeting Report from the 2023 McMaster ITP Summit.
Thromb Haemost
January 2025
Michael G. DeGroote Centre for Transfusion Research, McMaster University, Hamilton, Ontario, Canada.
The McMaster Immune Thrombocytopenia (ITP) Summit, held on October 27, 2023, was an educational seminar from leading experts in immune thrombocytopenia and related disorders geared toward hematologists, internists, immunologists, and clinical and translational scientists. The focus of the Summit was to review the mechanisms, diagnosis, and treatment of primary versus secondary ITP. Specific objectives were to describe the unique features of secondary ITP, and to review its mechanisms in the context of autoimmune disease and infection.
View Article and Find Full Text PDFTherapeutic Plasma Exchange Management for a Pediatric Patient Presenting With Immune Thrombotic Thrombocytopenic Purpura in a Setting of Common Variable Immunodeficiency.
J Clin Apher
December 2024
Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
Common variable immunodeficiency (CVID) is a disorder characterized by hypogammaglobulinemia resulting in recurrent infections. While autoimmune disorders are common in patients with CVID, no association has been reported between CVID and immune thrombotic thrombocytopenic purpura (iTTP), a disorder most often caused by autoantibodies that compromise the activity of the enzyme ADAMTS13. Reduced ADAMTS13 activity results in the accumulation of large von Willebrand factor multimers that can consume platelets and cause microvascular thrombosis and organ injury, ultimately resulting in mortality in most cases of untreated iTTP.
View Article and Find Full Text PDFExpanding the Clinical Phenotype with CD79A Mutation and Refractory Helicobacter Bilis Infection.
J Clin Immunol
August 2024
Allergy Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, 160012, India.
Article Synopsis
- * An 8-year-old boy with this disorder had a specific genetic mutation in the CD79A gene and developed difficult-to-treat leg ulcers caused by Helicobacter bilis, despite regular treatments like intravenous immunoglobulin and antibiotics.
- * The case highlights the necessity of accurate genetic diagnosis and specialized antimicrobial treatments when dealing with infections in patients with primary immunodeficiencies, especially those linked to CD79A mutations.
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