A Case of Haemophilic Arthropathy Seen in UTH Morafeno Toamasina

Hoby Nomena Rakotomalala, Randriamampianina Tahianasoa, Rakotoarivelo Zolalalaina Hubertine, Ralandison Stéphane, Rakoto Alson Aimée Olivat

Abstract


Introduction: Hemophilia is a hemorrhagic disease caused by congenital deficiency of factor VIII (hemophilia A) or factor IX (haemophilia B) coagulation. It is an inherited X-linked genetic inheritance disorder that affects mainly boys. Haemarthrosis is one of the common haemorrhagic signs of hemophilia, which could be complicated by advanced arthropathy that may affect the patient's functional prognosis. Our aim is to report through a case these rheumatologic complications of a haemophilia.

Observation: A 15-year-old boy with severe haemophilia, presenting recurrent hemarthrosis since the age of 10 years. He was seen for arthritis of the right knee which has deteriorated progressively over two years, with knee joint swelling, patellofemoral syndrome, an axial valgus deviation and a flessum of the knee, accompagnied by an amyotrophy of the quadriceps. Radiological examination showed stage III hemophilic arthropathy. He received substitution treatment with symptomatic treatments and physiotherapy.

Discussion and conclusion: Hemophilic arthropathy involves the functional prognosis of patients with severe hemophilia. Prophylactic replacement therapy has significantly decreased the frequency of advanced stages. However, in low-income countries, this prophylaxis is still limited because of the lack of accessibility and disponibility of replacement factor. There is a relatively broad therapeutic panel for the management of hemophilic arthropathy ranging from physiotherapy, to inflatrative procedures to surgery.


Keywords


Hemophilia, rheumatologic complication, arthropathy, prophylaxis, replacement therapy

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References


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