Download full-text PDF |
Source |
|---|
Publication Analysis
Top Keywords
Similar Publications
Characteristics of large granular lymphocyte leukemia associated with variable common immunodeficiency disorders: A study of 12 cases.
Eur J Haematol
October 2024
Internal Medicine and Infectious diseases, Hôpital Haut-Lévêque, Pessac, France.
Objectives: Common Variable Immunodeficiency Disorders (CVID) and Large Granular Lymphocytes leukemia (LGLL) exhibit diverse clinical manifestations including infections, dysimmunity, and lymphoproliferation. Recent decades have seen the discovery of new genes in the lymphopoiesis pathway, such as JAK-STAT. This case series supplemented by a literature review aims to describe clinical and biological characteristics of patients with both CIVD and LGLL.
View Article and Find Full Text PDFArticle Synopsis
- - The study investigates the link between eosinophilia and ocular vascular issues, highlighting the procoagulant effects of eosinophils, which can lead to various cardiovascular problems and eye conditions.
- - A retrospective, multicenter analysis of 57 patients revealed that the majority experienced eye issues as the first sign of eosinophil-related diseases, with specific conditions like central retinal artery occlusion being common among them.
- - The findings indicate a poor visual prognosis for these patients, with only 10% achieving full recovery, suggesting the need for urgent treatment and further research on eosinophilia's impacts.
[Dysimmune manifestations associated with myelodysplastic neoplasms and chronic myelomonocytic leukaemias].
Bull Cancer
November 2023
Sorbonne université, Assistance publique-Hôpitaux de Paris, centre hospitalo-universitaire Saint-Antoine, service de médecine interne et Inflammation-Immunopathology-Biotherapy Department (DMU i3), Paris, France. Electronic address:
Systemic inflammatory or autoimmune diseases (SIAD) are observed in up to a quarter of patients with myelodysplastic syndromes (MDS) or chronic myelomonocytic leukemia (CMML), with a broad clinical spectrum including asymptomatic biological abnormalities, isolated inflammatory clinical manifestations (recurrent fever, arthralgia, neutrophilic dermatoses…) or identified systemic diseases (giant cell arteritis, recurrent polychondritis…). Recent advances in molecular biology have shed new light on the pathophysiological mechanisms that link inflammatory manifestations and myeloid hemopathies, particularly in VEXAS syndrome following the identification of somatic mutations in the UBA1 gene, or in neutrophilic dermatoses with the concept of myelodysplasia cutis. Although the presence of SIAD does not seem to affect overall survival or the risk of transformation into acute myeloid leukemia, their treatment remains a challenge given the frequent high level of corticosteroid dependence as well as the poor efficacy and tolerance (cytopenias, infections) of conventional immunosuppressive agents.
View Article and Find Full Text PDFStiff person spectrum disorder diagnosis, misdiagnosis, and suggested diagnostic criteria.
Ann Clin Transl Neurol
July 2023
Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
Background: Stiff person spectrum disorder (SPSD) is heterogeneous, and accurate diagnosis can be challenging.
Methods: Patients referred for diagnosis/suspicion of SPSD at the Mayo Autoimmune Neurology Clinic from July 01, 2016, to June 30, 2021, were retrospectively identified. SPSD diagnosis was defined as clinical SPSD manifestations confirmed by an autoimmune neurologist and seropositivity for high-titer GAD65-IgG (>20.
Oculomotor nerve palsy as an extraintestinal manifestation of Crohn's disease.
BMJ Case Rep
May 2023
Department of Neurology, Hygeias Melathron, Athens, Greece.
Patients with inflammatory bowel disease (IBD) may present with extraintestinal manifestations. Neurological symptoms associated with IBD are infrequent. Thus, any unexplained neurological symptom that occurs in patients with IBD should raise the suspicion of a link between the two disorders.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!