13 research outputs found

    Extracorporeal photopheresis did not prevent the development of an autoimmune disease: myasthenia gravis

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    WOS: 000390578300026PubMed ID: 27612294BACKGROUNDMyasthenia gravis (MG) is a neuromuscular disorder characterized by an autoimmune defect in the neuromuscular junction. In most patients, the autoimmune attack is mediated by antibodies against the acetylcholine receptor (AChR) on the postsynaptic membrane. Deficient immunoregulation, including regulatory T cells, is consistently observed. Extracorporeal photopheresis (ECP) leads to the induction of regulatory T cells that mediate immunologic tolerance in autoimmune diseases; however, the data regarding MG are very limited. CASE REPORTHere, we report a patient who, during ongoing ECP therapy for his severe, refractory, chronic graft-versus-host disease (cGVHD), developed MG, although he responded very well to ECP, as indicated by the lowering of his chronic cGVHD severity grade to moderate. RESULTSDespite receiving ECP, our patient developed MG, which was resistant to treatment and required intensive care unit support. CONCLUSIONSClose surveillance is required when ECP is planned as one of the treatment alternatives in myasthenia gravis that develop in cGVHD

    Outcomes of high-grade gastrointestinal graft-versus-host disease posthematopoietic stem cell transplantation in children

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    WOS: 000388566200044PubMed ID: 27858879We explored the clinical course of acute high-grade gastrointestinal graft-versus-host disease in children in a single center. This was a retrospective analysis of 28 pediatric patients who presented with a clinical diagnosis of stage III and IV acute graft-versus-host disease (aGVHD) of the gastrointestinal system (GIS). Generally, skin involvement was the initial manifestation of aGVHD that began in the first 3 weeks of hematopoietic stem cell transplantation (HSCT); on the other hand, GIS involvement predominated after the second week of HSCT. Reported adult data show a survival rate of only 25%; however, our study showed more favorable outcomes in children with a survival rate of 55%. We monitored levels of albumin and immunoglobulin G and observed low levels overall during treatment of unresponsive patients, although only albumin levels were shown to be significantly different. We observed a significant increase in mortality with the use of antithymocyte globulin in GIS aGVHD, although antithymocyte globulin used for graft-versus-host disease prophylaxis had no demonstrable effect on GIS aGVHD mortality. Whether the significantly lower GIS aGVHD mortality among the children recruited in our study than among their historical adult counterparts is a primary result of the specific attributes of the pediatric GIS, or whether it originated from HSCT kinetics remains to be determined by future studies

    Outcomes of high grade gastrointestinal GVHD POST-HSCT in children

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    21st Congress of the European-Hematology-Association -- JUN 09-12, 2016 -- Copenhagen, DENMARKWOS: 000379484603291…The European Hematology Associatio
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