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Allograft loss in renal transplant recipients with Fabry's disease and activated protein C resistance.

Authors :
Friedman GS
Wik D
Silva L
Abdou JC
Meier-Kriesche HU
Kaplan B
Bonomini L
DeFranco P
Lyman N
Mulgaonkar S
Jacobs M
Source :
Transplantation [Transplantation] 2000 May 27; Vol. 69 (10), pp. 2099-102.
Publication Year :
2000

Abstract

Introduction: Fabry's disease is associated with an increased incidence of thrombotic events and rejection. Spontaneous thrombosis of a functioning cadaveric renal allograft in a recipient with Fabry's disease prompted prospective evaluation of all transplant candidates with Fabry's disease for hypercoagulability.<br />Materials and Methods: Transplant candidates with Fabry's disease were tested for hypercoagulability, analyzed for HLA-type and ABO group, and comorbid conditions suggestive of hypercoagulability.<br />Results: A unique association of Fabry's disease with activated protein C Resistance was documented in a cohort of Caucasian male renal transplant recipients with Fabry's disease. Four of five patients were blood group A and had no significant comorbid conditions suggestive of hypercoagulability. The resistance to activation of protein C (APCR)(+) patients shared HLA loci-B8 and Dr3, although the APCR(-) patients shared HLA loci-B27 and -B38.<br />Conclusions: Due to the observed increase in the incidence of APCR in our Fabry's cohort, we suggest screening all patients with Fabry's disease for APCR. Because factor V and factor Va receptors are found on vascular endothelium and peripheral blood monocytes, APCR in the presence of Fabry's disease may be a nonimmunological stimulus for rejection. Analysis of HLA typing in patients with Fabry's disease may further elucidate HLA-based association of Fabry's disease and resistance to activated protein C with the risk of thrombosis and rejection.

Details

Language :
English
ISSN :
0041-1337
Volume :
69
Issue :
10
Database :
MEDLINE
Journal :
Transplantation
Publication Type :
Academic Journal
Accession number :
10852604
Full Text :
https://doi.org/10.1097/00007890-200005270-00022