Clinical, instrumental, serological and histological findings suggest that hemophilia B may be less severe than hemophilia A
Daniela Melchiorre,
Silvia Linari,
Mirko Manetti,
Eloisa Romano,
Francesco Sofi,
Marco Matucci-Cerinic,
Christian Carulli,
Massimo Innocenti,
Lidia Ibba-Manneschi,
Giancarlo Castaman
Affiliations
Daniela Melchiorre
Department of Experimental and Clinical Medicine, Section of Internal Medicine, University of Florence, Rheumatology Unit, Careggi University Hospital, Florence, Italy
Silvia Linari
Center for Bleeding Disorders, Careggi University Hospital, Florence, Italy
Mirko Manetti
Department of Experimental and Clinical Medicine, Section of Anatomy and Histology, University of Florence, Florence, Italy
Eloisa Romano
Department of Experimental and Clinical Medicine, Section of Internal Medicine, University of Florence, Rheumatology Unit, Careggi University Hospital, Florence, Italy
Francesco Sofi
Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy;Don Carlo Gnocchi Foundation, Onlus IRCCS, Florence, Italy
Marco Matucci-Cerinic
Department of Experimental and Clinical Medicine, Section of Internal Medicine, University of Florence, Rheumatology Unit, Careggi University Hospital, Florence, Italy
Christian Carulli
First Orthopedic Clinic, Careggi University Hospital, Florence, Italy
Massimo Innocenti
First Orthopedic Clinic, Careggi University Hospital, Florence, Italy
Lidia Ibba-Manneschi
Department of Experimental and Clinical Medicine, Section of Anatomy and Histology, University of Florence, Florence, Italy
Giancarlo Castaman
Center for Bleeding Disorders, Careggi University Hospital, Florence, Italy
Recent evidence suggests that patients with severe hemophilia B may have a less severe disease compared to severe hemophilia A. To investigate clinical, radiological, laboratory and histological differences in the arthropathy of severe hemophilia A and hemophilia B, 70 patients with hemophilia A and 35 with hemophilia B with at least one joint bleeding were consecutively enrolled. Joint bleedings (50), regimen of treatment (prophylaxis/on demand), World Federation of Hemophilia, Pettersson and ultrasound scores, serum soluble RANK ligand and osteoprotegerin were assessed in all patients. RANK, RANK ligand and osteoprotegerin expression was evaluated in synovial tissue from 18 hemophilia A and 4 hemophilia B patients. The percentage of patients with either 10–50 or more than 50 hemarthrosis was greater in hemophilia A than in hemophilia B (P
