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  Highly skewed T-cell receptor V-beta chain repertoire in the bone marrow is associated with response to immunosuppressive drug therapy in children with very severe aplastic anemia

Schuster, F. R., Hubner, B., Fuehrer, M., Eckermann, O., Gombert, M., Dornmair, K., et al. (2011). Highly skewed T-cell receptor V-beta chain repertoire in the bone marrow is associated with response to immunosuppressive drug therapy in children with very severe aplastic anemia. BLOOD CANCER JOURNAL, 1: e8. doi:10.1038/bcj.2011.6.

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Item Permalink: http://hdl.handle.net/11858/00-001M-0000-000F-3D49-C Version Permalink: http://hdl.handle.net/11858/00-001M-0000-000F-3D4A-A
Genre: Journal Article

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 Creators:
Schuster, F. R., Author
Hubner, B., Author
Fuehrer, M., Author
Eckermann, O., Author
Gombert, M., Author
Dornmair, Klaus1, Author              
Binder, V., Author
Reuther, S., Author
Krell, P., Author
Keller, T., Author
Borkhardt, A., Author
Affiliations:
1Department: Neuroimmunology / Wekerle, MPI of Neurobiology, Max Planck Society, ou_1113547              

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Free keywords: IMMUNE-RESPONSES; PATHOPHYSIOLOGY; RECONSTITUTION; SURVIVAL; DISEASEaplastic anemia; T-cell receptor V-beta chain repertoire; children;
 Abstract: One of the major obstacles of immunosuppressive therapy (IST) in children with severe aplastic anemia (SAA) comes from the often months-long unpredictability of bone-marrow (BM) recovery. In this prospective study in children with newly diagnosed very severe AA (n = 10), who were enrolled in the therapy study SAA-BFM 94, we found a dramatically reduced diversity of both CD4+ and CD8+ BM cells, as scored by comprehensive V-beta chain T-cell receptor (TCR) analysis. Strongly skewed TCR V-beta pattern was highly predictive for good or at least partial treatment response (n = 6, CD8+ complexity scoring median 35.5, range 24-73). In contrast, IST in patients with rather moderate reduction of TCR V-beta diversity (n = 4, CD8+ complexity scoring median 109.5, range 82-124) always failed (P = 0.0095). If confirmed in a larger series of patients, TCR V-beta repertoire in BM may help to assign children with SAA up-front either to IST or to allogeneic stem-cell transplantation. Blood Cancer Journal (2011) 1, e8; doi:10.1038/bcj.2011.6; published online 4 March 2011

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Language(s): eng - English
 Dates: 2011-03-04
 Publication Status: Published online
 Pages: 4
 Publishing info: -
 Table of Contents: -
 Rev. Type: -
 Identifiers: ISI: 000298815400002
DOI: 10.1038/bcj.2011.6
 Degree: -

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Title: BLOOD CANCER JOURNAL
Source Genre: Journal
 Creator(s):
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Publ. Info: MACMILLAN BUILDING, 4 CRINAN ST, LONDON N1 9XW, ENGLAND : NATURE PUBLISHING GROUP
Pages: - Volume / Issue: 1 Sequence Number: e8 Start / End Page: - Identifier: ISSN: 2044-5385