TY - JOUR
T1 - Clinical, instrumental, serological and histological findings suggest that hemophilia b may be less severe than hemophilia a
AU - Melchiorre, Daniela
AU - Linari, Silvia
AU - Manetti, Mirko
AU - Romano, Eloisa
AU - Sofi, Francesco
AU - Matucci-Cerinic, Marco
AU - Carulli, Christian
AU - Innocenti, Massimo
AU - Ibba-Manneschi, Lidia
AU - Castaman, Giancarlo
PY - 2016/2/1
Y1 - 2016/2/1
N2 - 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
AB - 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
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U2 - 10.3324/haematol.2015.133462
DO - 10.3324/haematol.2015.133462
M3 - Article
AN - SCOPUS:84957614473
VL - 101
SP - 219
EP - 225
JO - Haematologica
JF - Haematologica
SN - 0390-6078
IS - 2
ER -