[Clinical management of patients with hemophilia A in nephrology: Diagnostic and therapeutic challenges illustrated by the cases of 2 patients]

Abstract

Hemophilia A is an X-linked genetic hemorrhagic disorder characterized by a factor VIII deficiency. The availability of secured substitution products has led to a dramatic improvement of life expectancy in hemophiliac patients. Nowadays, adult hemophiliac patients may develop Chronic Kidney Disease (CKD) resulting from age-related comorbidities (hypertension, obesity, diabetes). In addition, the high prevalence of viral infections in this population exposes patients to an increased risk of CKD. The risk of hemorrhage in hemophiliac patients is a challenge for their clinical management, both for diagnostic procedures (kidney biopsy in particular) and for renal replacement therapy (dialysis or renal transplantation) when it is needed. This work provides an update of the literature data concerning the management of hemophiliac patients in nephrology, illustrated by the cases of two patients.

Keywords: Biopsie rénale; Chronic kidney disease; Dialyse péritonéale; Hemodialysis; Hemophilia A; Hemorrhagic risk; Hémodialyse; Hémophilie A; Maladie rénale chronique; Peritoneal dialysis; Renal biopsy; Risque hémorragique; Transplantation.