Haemophilic Arthropathy Case Summary

Decent Essays
Haemophilic Arthropathy: Case Report

Liew Chiu Yee

Haemophilia arthropathy is a common complication seen in moderate and severe haemophilic patient due to recurrent haemarthrosis. It is often begins in first or second decade of life and bleeding tend to recur in the same joint with gradual progression to joint destruction. Prophylactic replacement of deficient clotting factors may prevent or delay joint destruction but in many countries factor replacement is on demand basis due to financial constraint. This is a case report illustrates a patient with moderate Haemophilia B with haemophilic arthropathy.

Case Report
A 33-year-old Chinses gentleman was diagnosed with moderate Haemophilia B at the age of 4. He was
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However there are 30% of cases are resulted from spontaneous mutation without any family history. Factor VIII deficiency (Haemophilia A) is the commonest type while incidence of Factor IX deficiency (Haemophilia B/ Christmas disease) is one fifth of it. Haemophilia is classified into 3 categories depending on the percentage of coagulation factor activity: severe (< 1%), moderate (2-5%) and mild (5-30%). Moderate and severe haemophilia has significant clinical manifestation as the patients have spontaneous bleeding especially into joint and…show more content…
Intra-articular bleeding leads to hypertrophic synovium as the normal synovium is unable to reabsorbed excessive blood within the joint. The hypertrophic synovium is hyperaemic and richly vascularized resulting in asymmetrical growth of the epiphyses lead to angular deformities and limb length discrepancies. If chronic haemophilic synovitis left untreated it will result in cartilage damage and lead to haemophilic arthropathy, a particular form degenerative osteoarthritis. The common joint deformity seen in haemophilia patients are equinus deformity of the foot, flexion contractures of the knees and elbows. Intramuscular haematoma complicated with nerve palsy and fibrosis also contribute to fixed articular contracture.
Patient with severe and moderate haemophilia can be managed with prophylactic factor replacement or on-demand therapy whenever bleeding occurred. A large retrospective randomized study over four decades showed that primary prophylaxis initiated at early age before onset of recurrent bleeding episodes reduces incidence of haemophilic arthropathy and lead to normal life (2).
Haemophilia can cause progressive joint destruction and loss of function due to recurrent haemarthrosis. Early prophylactic factor replacement in moderate and severe haemophilia patient can prevent or delay the joint destruction however cost of the treatment is an issue need to be considered as this
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