Haemophilia B: clinical manifestations and complications

ABSTRACT

Haemophilia B is X-linked recessive inherited disorder of factor IX deficiency. It is classified as severe, moderate and mild depending upon plasma levels of factor IX. The development of inhibitors is seen during treatment of haemophilia B against F-IX. This study was aimed to determine the frequency of different complications in haemophilia B patients. Total 45 patients of Haemophilia B already enrolled in the Haemophilia society of Pakistan Lahore chapter were included in this study. Clinical history and physical examinations were recorded on a pre designed proforma. Laboratory testing for establishment of diagnosis of haemophilia B and inhibitors of FIX was done. Out of 45 patients, 10 [22.2%] had severe disease while 28 [62.2%] had moderate and 07 [15.6%] had mild disease. Twenty nine [64.4%] of patients with severe and moderate disease were diagnosed below 5 years of age while none with mild disease was diagnosed under 5 years of age. Arthropathy was the most frequently developing complication in patients 10 [100%] of severe Hemophilia B. Post circumcision bleeding was found to be the most common first episode of bleeding in patients of haemophilia B 29 [64.4%]. Inhibitor against F-IX developed in only one patient of severe disease 1 [10%]. Arthropathy is the commonest complication and circumcision is the first bleeding site in most of the haemophiliacs