Changing patterns of bleeding in patients with severe haemophilia A

Stephensen, David; Tait, R. C.; Brodie, N.; Collins, Peter William; Cheal, R.; Keeling, David; Melton, Kristin; Dolan, G.; Haye, H.; Hayman, E.; Winter, Mark

doi:10.1111/j.1365-2516.2008.01876.x

Cited by 124 publications

(144 citation statements)

References 9 publications

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“…Although bleeding may occur anywhere, approximately 70–80% of haemorrhages occurs in the joints, spontaneously as well as in response to mechanical stress or trauma,3 4 causing joint degeneration by inflammation and direct tissue damage, called haemophilic arthropathy (HA) 5 6. Iron is hypothesised to be important in inducing arthropathy in HH and HA, although in both diseases the exact pathogenesis has not been fully elucidated.…”

Section: Introductionmentioning

confidence: 99%

The detrimental effects of iron on the joint: a comparison between haemochromatosis and haemophilia

et al. 2015

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Joint damage due to (recurrent) joint bleeding in haemophilia causes major morbidity. Although the exact pathogenesis has not been fully elucidated, a central role for iron is hypothesised. Likewise, in hereditary haemochromatosis joint destruction is caused by iron overload. A comparison between these types of arthropathy could provide more insight in the influence of iron in inducing joint damage. A literature review was performed to compare both disorders with respect to their clinical and histological characteristics, and preclinical studies on the influence of iron on different joint components were reviewed. Similarities in the features of arthropathy in haemochromatosis and haemophilia are cartilage degeneration, subchondral bone changes with osteophyte and cyst formation, and osteoporosis. In both disorders synovial inflammation and proliferation are seen, although this is much more explicit in haemophilia. Other substantial differences are the age at onset, the occurrence of chondrocalcinosis radiographically and calcium pyrophosphate dihydrate deposition disease in haemochromatosis, and a rapid progression with joint deformity and neovascularisation in haemophilia. Preclinical studies demonstrate detrimental effects of iron to all components of the joint, resulting in synovial inflammation and hyperplasia, chondrocyte death, and impaired osteoblast function. These effects, particularly the synovial changes, are aggravated in the presence of a pro-inflammatory signal, which is prominent in haemophilic arthropathy and minimal in haemochromatosis. Additional research is needed to further specify the role of iron as a specific target in treating these types of arthropathy.

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Section: Introductionmentioning

confidence: 99%

The detrimental effects of iron on the joint: a comparison between haemochromatosis and haemophilia

et al. 2015

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“…Bleeds can be due to trauma or apparently occur spontaneously 3. A child with severe haemophilia can present during the first year of life with bleeding following surgical intervention such as circumcision, intramuscular immunisations or rarely ‘spontaneously’ with intracranial haemorrhage4 Often the child will have no problems until they start to mobilise around 18–24 months when ‘pea-sized’ lumpy bruises are commonly seen.…”

Section: Presentation and Diagnosismentioning

confidence: 99%

Major bleeding disorders: diagnosis, classification, management and recent developments in haemophilia

Bhatnagar

Hall

2017

Arch Dis Child

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In this review, we outline the standard of care for children in the UK with the most common major bleeding disorder, haemophilia, and how exciting new developments in therapy have the potential for further improvements in quality of life and clinical outcome. The combination of comprehensive specialist medical care, safer factor concentrates, earlier introduction of prophylaxis and patient-specific education has allowed the current generation of patients with haemophilia to grow into adulthood with excellent joint function, pursuing full-time employment with a good quality of life. We are entering an exciting new phase in paediatric haemophilia as potentially life-changing products appear on the scene taking a step towards achieving better, easier and personalised prophylaxis.

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“…When synovitis becomes chronic, the condition is worsened by the recurrent haemarthrosis episodes, accelerating the degenerative process known as haemophilic arthropathy, which leads to alterations of the joints, pain, muscular atrophy, and functional impairment [16]. Chronic arthropathy is a major cause of morbidity in patients with haemophilia [17].…”

Section: Introductionmentioning

confidence: 99%

Physiotherapy Treatment in Patients with Hemophilia and Chronic Ankle Arthropathy: A Systematic Review

Cuesta‐Barriuso

Gómez–Conesa

López‐Pina

2013

Rehabilitation Research and Practice

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Haemophilic arthropathy of the ankle causes pain and deterioration in gait, causing disability. Although some physiotherapy modalities are effective in the management of acute bleeding, the results are unknown in chronic arthropathy. Our objective was to determine the most effective physiotherapy procedures for treating the haemophilic arthropathy of the ankle and to assess the methodological quality of the studies. A systematic review was carried out in the Cochrane Database, PubMed, MEDLINE, ISI Web of Knowledge, PEDro, TESEO, and specialized journals (Haemophilia and Haematologica). It included articles with at least one group undergoing any kind of physiotherapy treatment and with pretest and posttest evaluation, published before April 2013. An analysis of variables was performed and assessed the methodological quality of studies. Five studies met the criteria for inclusion. Hydrotherapy treatments, strength training and balance strength, balance training, and sports therapy, have improved range of movement, pain, balance, and subjective physical performance. The proposed methodological analysis was not possible due to the low quality of the studies. Although the results are positive, they lack rigorous evidence on the effects of treatments. Studies are needed to establish the efficacy of the various forms of physiotherapy in the haemophilic arthropathy of the ankle.

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Changing patterns of bleeding in patients with severe haemophilia A

Cited by 124 publications

References 9 publications

The detrimental effects of iron on the joint: a comparison between haemochromatosis and haemophilia

The detrimental effects of iron on the joint: a comparison between haemochromatosis and haemophilia

Major bleeding disorders: diagnosis, classification, management and recent developments in haemophilia

Physiotherapy Treatment in Patients with Hemophilia and Chronic Ankle Arthropathy: A Systematic Review

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