A Study of Haemophilic Patient's Indication for admission and early detection of Arthropathy

INTRODUCTION : Haemophilia is a disorder of coagulation. Haemophilia A is due to deficiency of clotting factor VIII and haemophilia B is due to deficiency of factor IX. Both are inherited as X-linked, so males usually exhibit the disease and females are carriers. Prevalence of haemophilia A is 1 in 5,000 to 10,000 male births and that of haemophilia B is 1 in 20,000 to 34,000 births. Haemophilia A accounts for about 80% cases of haemophilia and haemophilia B around 20%. Arthropathy is a common and debilitating complication of haemophilia. If detected earlier progression of disease process can be arrested by effective management. METHODS : Here 40 patients with haemophilia are studied and their indications of admissions were noted. They were assessed clinically as well as radiologically for the detection of arthropathy irrespective of whether they were admitted for musculoskeletal bleeds or not. RESULTS : Majority of patients were admitted with musculoskeletal bleeds(29 of 40). Most of the patients were haemophilia A patients(34 of 40). Family history was present in only 10% cases. Majority of patients(23 of 40) were in stage III of haemophilic arthropathy as staged according to Arnold hilgartner classification. Out of 11 patients admitted with non musculoskeletal bleeds only one had clinical evidence of arthropathy and out of the remaining 10 patients 4 were already in stage III of haemophilic arthropathy without any symptoms and signs. Arthropathy worsens as the duration of disease process increases. CONCLUSIONS : Haemophilia is a common coagulation disorder encountered by physicians. The most common indication for admissions are musculoskeletal bleeds and knee being the commonest joint involved. As the duration of disease process increases the risk of permanent joint damage also goes on the increase. Those patients with early stages of arthropathy are treated conservatively with factor replacement therapy to halt the progression of disease. If not treated adequately, due to chronic synovitis permanent joint damage occurs. Once this happens only surgical cure is possible to restore the joint function. The various modalities available are synovectomy, arthroplasty and joint replacement in severe cases. Even in haemophilics admitted for non-musculoskeletal indications arthropathy may lie undetected and if unnoticed can result in joint damage.