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Acquired factor XIII deficiency in myeloid neoplasms: case series and review of literature.
Hematol Oncol Stem Cell Ther
January 2025
Adult Hematology, Stem Cell Transplant and Cellular Therapy Section, Oncology Center, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
Acquired factor XIII (FXIII) deficiency is a rare disorder that could be associated with autoimmune and malignant disorders with a high risk of bleeding. In acute leukemias, acquired FXIII deficiency has been reported and replacement of FXIII helped to control significant bleeding. Here, we report four cases of myeloid neoplasms to have acquired FXIII deficiency with interesting concomitant RUNX1 mutation in the molecular background of two patients.
View Article and Find Full Text PDFHypomethylating agents for patients with VEXAS without myelodysplastic syndrome: Clinical outcome and longitudinal follow-up of vacuolization and UBA1 clonal dynamics.
Br J Haematol
January 2025
Institut d'Investigacions Biomèdiques August Pi iSunyer (IDIBAPS), Barcelona, Spain.
VEXAS syndrome is a haemato-inflammatory disease caused by somatic UBA1 mutations and characterized by cytoplasmic vacuoles in myeloid and erythroid precursor cells. Although there is currently no standard treatment algorithm for VEXAS, patients are generally treated with anti-inflammatory therapies focused on symptom management, with only partial effectiveness. Hypomethylating agents (HMA) have shown promise in VEXAS patients with concomitant myelodysplastic syndrome (MDS), while the efficacy of HMA in VEXAS patients without MDS is largely unknown.
View Article and Find Full Text PDFC5 complement inhibition versus FcRn modulation in generalised myasthenia gravis.
J Neurol Neurosurg Psychiatry
January 2025
Department of Neurology, Medical Faculty, Heinrich Heine University Düsseldorf, Düsseldorf, Germany
Background: Myasthenia gravis (MG) is an autoimmune disorder affecting neuromuscular junctions, leading to fluctuating muscle weakness. While many patients respond well to standard immunosuppression, a substantial subgroup faces ongoing disease activity. Emerging treatments such as complement factor C5 inhibition (C5IT) and neonatal Fc receptor (FcRn) antagonism hold promise for these patients.
View Article and Find Full Text PDFImmediate management of a cirrhosis-induced severe pericardial effusion: a case report and review of the literature.
J Med Case Rep
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Cardiac Surgery Department, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Introduction: Cardiac tamponade is a life-threatening condition resulting from fluid accumulation in the pericardial sac, leading to decreased cardiac output and shock. Various etiologies can cause cardiac tamponade, including liver cirrhosis, which may be induced by autoimmune hepatitis. Autoimmune hepatitis is a chronic inflammatory liver disease characterized by interface hepatitis, elevated transaminase levels, autoantibodies, and increased immunoglobulin G levels.
View Article and Find Full Text PDFThe profile of oxidative stress markers (arachidonic and linoleic acid derivatives) in patients with benign prostatic hyperplasia in relation to metabolic syndrome.
Aging (Albany NY)
January 2025
Department of Obstetrics and Pathology of Pregnancy, Pomeranian Medical University, Żołnierska, Szczecin 71-210, Poland.
So far, it has been proven that benign prostatic hyperplasia (BPH) is strongly associated with inflammation resulting from, i.a. the presence of infectious agent, autoimmune disease, aging process and lipid disorders associated with metabolic syndrome (MetS).
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