Clinical, instrumental, serological and histological findings suggest that hemophilia B may be less severe than hemophilia A

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...

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Published in:Haematologica (Roma) Vol. 101; no. 2; pp. 219 - 225
Main Authors: Melchiorre, D., Linari, S., Manetti, M., Romano, E., Sofi, F., Matucci-Cerinic, M., Carulli, C., Innocenti, M., Ibba-Manneschi, L., Castaman, G.
Format: Journal Article
Language:English
Published: Italy Ferrata Storti Foundation 01.02.2016
Subjects:
Adolescent
Adult
Aged
Child
Child, Preschool
Disease Progression
Female
Gene Expression
Hemarthrosis - complications
Hemarthrosis - diagnostic imaging
Hemarthrosis - genetics
Hemarthrosis - pathology
Hemophilia A - complications
Hemophilia A - diagnostic imaging
Hemophilia A - genetics
Hemophilia A - pathology
Hemophilia B - complications
Hemophilia B - diagnostic imaging
Hemophilia B - genetics
Hemophilia B - pathology
Humans
Joint Capsule - chemistry
Joint Capsule - pathology
Male
Middle Aged
Osteoprotegerin - blood
Osteoprotegerin - genetics
RANK Ligand - blood
RANK Ligand - genetics
Receptor Activator of Nuclear Factor-kappa B - blood
Receptor Activator of Nuclear Factor-kappa B - genetics
Severity of Illness Index
Ultrasonography
ISSN:0390-6078, 1592-8721, 1592-8721
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Summary: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 (<10, 10-50, >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<0.001 and P=0.03, respectively), while that with less than 10 hemarthrosis was higher in hemophilia B (P<0.0001). World Federation of Hemophilia (36.6 vs. 20.2; P<0.0001) and ultrasound (10.9 vs. 4.3; P<0.0001) score mean values were significantly higher in hemophilia A patients. Serum osteoprotegerin and soluble RANK ligand were decreased in hemophilia A versus hemophilia B (P<0.0001 and P=0.006, respectively). Osteoprotegerin expression was markedly reduced in synovial tissue from hemophilia A patients. In conclusion, the reduced number of hemarthrosis, the lower World Federation of Hemophilia and ultrasound scores, and higher osteoprotegerin expression in serum and synovial tissue in hemophilia B suggest that hemophilia B is a less severe disease than hemophilia A. Osteoprotegerin reduction seems to play a pivotal role in the progression of arthropathy in hemophilia A.
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LI-M and GC contributed equally to this work.
ISSN:0390-6078
1592-8721
1592-8721
DOI:10.3324/haematol.2015.133462