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


METHODS
Transplant candidates with Fabry's disease were tested for hypercoagulability, analyzed for HLA-type and ABO group, and comorbid conditions suggestive of hypercoagulability.


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.


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.
Friedman
G S
GS
Saint Barnabas Medical Center, Department of Transplantation, Livingston, New Jersey 07039, USA.
Wik
D
D
Silva
L
L
Abdou
J C
JC
Meier-Kriesche
H U
HU
Kaplan
B
B
Bonomini
L
L
DeFranco
P
P
Lyman
N
N
Mulgaonkar
S
S
Jacobs
M
M
D000017:ABO Blood-Group System; D020016:Activated Protein C Resistance / Q000150:complications* / Q000276:immunology; D000328:Adult; D015331:Cohort Studies; D015897:Comorbidity; D000795:Fabry Disease / Q000150:complications* / Q000276:immunology; D015235:HLA-B Antigens / Q000032:analysis; D015796:HLA-B27 Antigen / Q000032:analysis; D059945:HLA-B38 Antigen; D015795:HLA-B8 Antigen / Q000032:analysis; D015803:HLA-DR3 Antigen / Q000032:analysis; D006650:Histocompatibility Testing; D006801:Humans; D016030:Kidney Transplantation / Q000276:immunology; D008297:Male; D008875:Middle Aged; D014184:Transplantation, Homologous; D017211:Treatment Failure; D044465:White People
D000017:ABO Blood-Group System; D015235:HLA-B Antigens; D015796:HLA-B27 Antigen; D059945:HLA-B38 Antigen; D015795:HLA-B8 Antigen; D015803:HLA-DR3 Antigen