Blood online
Home About Blood Authors Subscriptions Permission Advertising Public Access contact us
 

 
Advanced
Current Issue
First Edition
Future Articles
Archives
Submit to Blood
Search
American Society of Hematology
Meeting Abstracts
Email Alerts
 Blood, 1 October 2005, Vol. 106, No. 7, pp. 2399-2408.
Prepublished online as a Blood First Edition Paper on June 14, 2005; DOI 10.1182/blood-2004-11-4315.

This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow All Versions of this Article:
2004-11-4315v1
106/7/2399    most recent
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrow reprints & permissions
Right arrow Rights and Permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Poggi, A.
Right arrow Articles by Notaro, R.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Poggi, A.
Right arrow Articles by Notaro, R.
Related Collections
Right arrow Hematopoiesis and Stem Cells
Right arrow Immunobiology
Right arrow Red Cells
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us   Add to Digg   Add to Reddit   Add to Technorati  
What's this?

arrow to previous article Previous Article  |  Table of Contents  |  Next Article next article arrow

IMMUNOBIOLOGY

Patients with paroxysmal nocturnal hemoglobinuria have a high frequency of peripheral-blood T cells expressing activating isoforms of inhibiting superfamily receptors

Alessandro Poggi, Simone Negrini, Maria Raffaella Zocchi, Anna-Maria Massaro, Lucia Garbarino, Sonia Lastraioli, Lucia Gargiulo, Lucio Luzzatto, and Rosario Notaro

From the Laboratory of Experimental Oncology and the Laboratory of Human Genetics, National Institute for Cancer Research, the Laboratory of Clinical Immunology, Department of Internal Medicine, University of Genoa, and the Laboratory of Hystocompatibility/IBMDR, Galliera Hospital, Genoa, Italy; and the Laboratory of Tumor Immunology, San Raffaele Scientific Institute, Milan, Italy.

Patients with paroxysmal nocturnal hemoglobinuria (PNH) have a large clonal population of blood cells deriving from hematopoietic stem cells (HSCs) deficient in glycosylphosphatidylinositol (GPI)-anchored surface molecules. A current model postulates that PNH arises through negative selection against normal HSCs exerted by autoreactive T cells, whereas PNH HSCs escape damage. We have investigated the inhibitory receptor superfamily (IRS) system in 13 patients with PNH. We found a slight increase in the proportion of T cells expressing IRS. In contrast to what applies to healthy donors, the engagement of IRS molecules on T cells from patients with PNH elicited a powerful cytolytic activity in a redirected killing assay, indicating that these IRSs belong to the activating type. This was confirmed by clonal analysis: 50% of IRS+ T-cell clones in patients with PNH were of the activating type, while only 5% were of the activating type in healthy donors. Moreover, the ligation of IRS induces (1) production of tumor necrosis factor {alpha} (TNF-{alpha}) and interferon {gamma} (IFN-{gamma}) and (2) brisk cytolytic activity against cells bearing appropriate IRS counter-ligands. In addition, these IRS+ T cells show natural killer (NK)-like cytolytic activity to which GPI- cells were less sensitive than GPI+ cells. Thus, T cells with NK-like features, expressing the activating isoforms of IRS, may include effector cells involved in the pathogenesis of PNH.


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us   Add to Digg Digg   Add to Reddit Reddit   Add to Technorati Technorati    What's this?


This article has been cited by other articles:


Home page
 BloodHome page
L. Gargiulo, S. Lastraioli, G. Cerruti, M. Serra, F. Loiacono, S. Zupo, L. Luzzatto, and R. Notaro
Highly homologous T-cell receptor beta sequences support a common target for autoreactive T cells in most patients with paroxysmal nocturnal hemoglobinuria
Blood, June 1, 2007; 109(11): 5036 - 5042.
[Abstract] [Full Text] [PDF]

Home page
 J. Immunol.Home page
J. Chen, F. M. Ellison, M. A. Eckhaus, A. L. Smith, K. Keyvanfar, R. T. Calado, and N. S. Young
Minor Antigen H60-Mediated Aplastic Anemia Is Ameliorated by Immunosuppression and the Infusion of Regulatory T Cells
J. Immunol., April 1, 2007; 178(7): 4159 - 4168.
[Abstract] [Full Text] [PDF]

Home page
 BloodHome page
N. S. Young, R. T. Calado, and P. Scheinberg
Current concepts in the pathophysiology and treatment of aplastic anemia
Blood, October 15, 2006; 108(8): 2509 - 2519.
[Abstract] [Full Text] [PDF]

Home page
 ASH Education BookHome page
N. S. Young
Pathophysiologic Mechanisms in Acquired Aplastic Anemia
Hematology, January 1, 2006; 2006(1): 72 - 77.
[Abstract] [Full Text] [PDF]


click for free articles
home about blood authors subscriptions permissions advertising public access contact us
  Copyright © 2005 by American Society of Hematology         Online ISSN: 1528-0020