Literature DB >> 18992055

Rapid full engraftment and successful immune reconstitution after allogeneic hematopoietic stem cell transplantation with reduced intensity conditioning in Omenn syndrome.

Jolanta Gozdzik1, Wojciech Czogala, Szymon Skoczen, Aleksandra Krasowska-Kwiecien, Oktawiusz Wiecha, Anna Mordel, Ewa Lesko, Marcin Majka, Danuta Kowalczyk, Marek Zembala.   

Abstract

OS is a variant of SCID characterized by generalized erythroderma, alopecia, eosinophilia, and elevated IgE levels. It is fatal unless treated with allogeneic HSCT, which is the only curative approach. However, treatment related complications and graft rejection are major obstacles to the success of treatment. In this report, we describe a patient with OS, complicated by prolonged cytomegalovirus infection, successfully treated by reduced intensity conditioning allogeneic HSCT from sibling donor.

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Year:  2008        PMID: 18992055     DOI: 10.1111/j.1399-3046.2008.01020.x

Source DB:  PubMed          Journal:  Pediatr Transplant        ISSN: 1397-3142


  2 in total

1.  Anti-CD3ε mAb improves thymic architecture and prevents autoimmune manifestations in a mouse model of Omenn syndrome: therapeutic implications.

Authors:  Veronica Marrella; Pietro L Poliani; Elena Fontana; Anna Casati; Virginia Maina; Barbara Cassani; Francesca Ficara; Manuela Cominelli; Francesca Schena; Marianna Paulis; Elisabetta Traggiai; Paolo Vezzoni; Fabio Grassi; Anna Villa
Journal:  Blood       Date:  2012-06-21       Impact factor: 22.113

2.  Analysis of Peripheral Blood Mononuclear Cells Gene Expression Highlights the Role of Extracellular Vesicles in the Immune Response following Hematopoietic Stem Cell Transplantation in Children.

Authors:  Wojciech Strojny; Kinga Kwiecińska; Przemysław Hałubiec; Wojciech Kowalczyk; Karol Miklusiak; Agnieszka Łazarczyk; Szymon Skoczeń
Journal:  Genes (Basel)       Date:  2021-12-17       Impact factor: 4.096

  2 in total

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