Two cases of splenic peliosis are reported. Clinical and radiographic signs and laboratory results do not contribute greatly to diagnosis which is based on histology findings. Splenic rupture is the major complication and requires immediate splenectomy.
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Exosomal miR-146a-5p derived from bone marrow mesenchymal stromal cells regulate Th1/Th2 balance and alleviates immune thrombocytopenia in pregnancy.
Hum Cell
December 2024
Department of Oncology, Donghu District, First Affiliated Hospital of Nanchang University, No. 17 Yongwaizheng Street, Nanchang, 330000, Jiangxi, China.
Immune thrombocytopenia (ITP) is a common hematological disorder. Our previous study has found that exosomal miR-146a-5p derived from bone marrow mesenchymal stromal cells (BMSCs) regulate Th17/Treg balance to alleviate ITP. This work further investigated the role of miR-146a-5p in ITP with pregnancy.
View Article and Find Full Text PDFCase report: Pediatric patient with severe clinical course of CTLA-4 insufficiency treated with HSCT.
Front Immunol
December 2024
Department of Pediatric Hematology, Oncology and Transplantology, Medical University of Lublin, Lublin, Poland.
Background: Cytotoxic T lymphocyte-associated antigen-4 (CTLA-4) insufficiency is a rare disease belonging to inborn errors of immunity. Most cases of patients with CTLA-4 insufficiency are diagnosed in adults, therefore it is not a common problem in the clinical practice of pediatricians. However, it is worth noticing that most cases described in the literature show the first symptoms of the disease before the age of 18, but the phenotypic variability of patients complicates and delays the diagnostic process.
View Article and Find Full Text PDFRecurrent Immune Thrombocytopenia After Liver Transplantation in a Pediatric Patient: Successful Bridging Treatment With Eltrombopag Before Partial Splenic Embolization and Review of the Literature.
Exp Clin Transplant
October 2024
From the Department of Pediatrics, Başkent University Faculty of Medicine, Ankara, Turkey.
Although thrombocytopenia is a frequent complication that develops after liver transplantation, immune thrombocytopenia rarely occurs post-transplant. We present an 8-year-old boy who experienced 2 immune thrombocytopenia episodes that occurred 72 and 93 months after liver transplant while the patient was on tacrolimus therapy. The patient, who had primary diagnosis being deoxyguanosine kinase deficiency, received a liver transplant from his father at 1 year of age.
View Article and Find Full Text PDFPlatelet Responses After Tapering and Discontinuation of Fostamatinib in Patients with Immune Thrombocytopenia: A Continuation of the Fostasur Study.
J Clin Med
October 2024
Hospital de la Serranía de Ronda, 29400 Málaga, Spain.
Article Synopsis
- Fostamatinib is an approved treatment for adults with chronic immune thrombocytopenia (ITP) but there's limited research on tapering doses and achieving remission after stopping the drug.
- A study involving 61 patients examined the safety and effectiveness of fostamatinib during and after dose tapering, noting that complete platelet response was typically achieved within 28 days.
- Results showed that most patients maintained platelet counts above 100 × 10/L after discontinuation, with a low relapse rate, suggesting that tapering could lead to sustained remission, though further research on tapering strategies is needed.
A novel small molecule phagocytosis inhibitor, KB-208, ameliorates ITP in mouse models with similar efficacy as IVIG.
Transfusion
December 2024
Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada.
Background: The characteristic feature of immune cytopenias involves the process of extravascular phagocytosis, wherein macrophages in the spleen and/or liver engage in the destruction of blood cells that have been opsonized by auto- or alloantibodies. Therefore, new treatments that prevent phagocytosis will be advantageous, especially for short-term usage along with alternative options.
Study Design And Methods: KB-208, a small molecule drug, previously shown to be efficacious for the in vitro inhibition of phagocytosis was synthesized.
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