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Vol 3, No 1 (2010)
Review paper
Published online: 2010-03-19
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Hemophilic arthropathy - pathogenesis, clinical symptoms and diagnostic possibilities

Piotr Żbikowski, Michał Matysiak, Paweł Łaguna, Jarosław Ćwikła
Journal of Transfusion Medicine 2010;3(1):19-26.

open access

Vol 3, No 1 (2010)
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Published online: 2010-03-19

Abstract

Hemophilic arthropathy is a fundamental clinical problem in patients with severe hemophilia, particularly in patients with inhibitor. Initial intraarticular bleeds occur in first years of life and concern mainly knee, elbow and ankle joints. Overgrowth of synovium and direct toxic effect of blood components on the cartilage are major underlying factors of arthropathy pathogenesis. Typical symptoms include intraarticular bleeds leading to gradual loss of range of motion, joint deformity, muscular atrophia, loss of function and limitation of patient’s activity. Radiological signs of hemophilic arthropathy comprise osteopenia, epiphyseal overgrowth, irregular subchondral layer, erosions and incongruity of articular surfaces, subluxation or/ and angular deformity of the joint. There is growing interest in the use of magnetic resonance imaging and ultrasonography in the diagnosis of early stages of hemophilic arthropathy.

Abstract

Hemophilic arthropathy is a fundamental clinical problem in patients with severe hemophilia, particularly in patients with inhibitor. Initial intraarticular bleeds occur in first years of life and concern mainly knee, elbow and ankle joints. Overgrowth of synovium and direct toxic effect of blood components on the cartilage are major underlying factors of arthropathy pathogenesis. Typical symptoms include intraarticular bleeds leading to gradual loss of range of motion, joint deformity, muscular atrophia, loss of function and limitation of patient’s activity. Radiological signs of hemophilic arthropathy comprise osteopenia, epiphyseal overgrowth, irregular subchondral layer, erosions and incongruity of articular surfaces, subluxation or/ and angular deformity of the joint. There is growing interest in the use of magnetic resonance imaging and ultrasonography in the diagnosis of early stages of hemophilic arthropathy.

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Keywords

hemophilia; arthropathy; joint; cartilage; pathogenesis; radiologic signs; clinical signs

About this article
Title

Hemophilic arthropathy - pathogenesis, clinical symptoms and diagnostic possibilities

Journal

Journal of Transfusion Medicine

Issue

Vol 3, No 1 (2010)

Article type

Review paper

Pages

19-26

Published online

2010-03-19

Bibliographic record

Journal of Transfusion Medicine 2010;3(1):19-26.

Keywords

hemophilia
arthropathy
joint
cartilage
pathogenesis
radiologic signs
clinical signs

Authors

Piotr Żbikowski
Michał Matysiak
Paweł Łaguna
Jarosław Ćwikła

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