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

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 hyp...

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Veröffentlicht in:Transplantation 2000-05, Vol.69 (10), p.2099-2102
Hauptverfasser: FRIEDMAN, G. S, WIK, D, JACOBS, M, SILVA, L, ABDOU, J. C, MEIER-KRIESCHE, H. U, KAPLAN, B, BONOMINI, L, DEFRANCO, P, LYMAN, N, MULGAONKAR, S
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container_end_page 2102
container_issue 10
container_start_page 2099
container_title Transplantation
container_volume 69
creator FRIEDMAN, G. S
WIK, D
JACOBS, M
SILVA, L
ABDOU, J. C
MEIER-KRIESCHE, H. U
KAPLAN, B
BONOMINI, L
DEFRANCO, P
LYMAN, N
MULGAONKAR, S
description 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. Transplant candidates with Fabry's disease were tested for hypercoagulability, analyzed for HLA-type and ABO group, and comorbid conditions suggestive of hypercoagulability. 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. 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.
doi_str_mv 10.1097/00007890-200005270-00022
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S ; WIK, D ; JACOBS, M ; SILVA, L ; ABDOU, J. C ; MEIER-KRIESCHE, H. U ; KAPLAN, B ; BONOMINI, L ; DEFRANCO, P ; LYMAN, N ; MULGAONKAR, S</creator><creatorcontrib>FRIEDMAN, G. S ; WIK, D ; JACOBS, M ; SILVA, L ; ABDOU, J. C ; MEIER-KRIESCHE, H. U ; KAPLAN, B ; BONOMINI, L ; DEFRANCO, P ; LYMAN, N ; MULGAONKAR, S</creatorcontrib><description>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. Transplant candidates with Fabry's disease were tested for hypercoagulability, analyzed for HLA-type and ABO group, and comorbid conditions suggestive of hypercoagulability. 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. 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. 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subjects ABO Blood-Group System
Activated Protein C Resistance - complications
Activated Protein C Resistance - immunology
Adult
Biological and medical sciences
Cohort Studies
Comorbidity
European Continental Ancestry Group
Fabry Disease - complications
Fabry Disease - immunology
Histocompatibility Testing
HLA-B Antigens - analysis
HLA-B27 Antigen - analysis
HLA-B38 Antigen
HLA-B8 Antigen - analysis
HLA-DR3 Antigen - analysis
Humans
Kidney Transplantation - immunology
Male
Medical sciences
Middle Aged
protein C
Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases
Surgery of the urinary system
Transplantation, Homologous
Treatment Failure
title Allograft loss in renal transplant recipients with Fabry's disease and activated protein C resistance
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