DOI: 10.36347/sjams.2017.v05i11.025

Pages: 4436-4440

Haemophilia is a group of related inherited bleeding disorders that include abnormalities of coagulation factors and platelet function and is a hereditary X-linked coagulation disorder caused by deficiency or reduced activity of Factor VIII in Haemophilia A or Factor IX in Haemophilia B. To assess the clinical profile of haemophilia patients in our region. The present study was conducted in Hematology section of Postgraduate Department of Pathology, Government Medical College Jammu. Patients of all age groups who were registered at comphrensive hemophilia care centre, Govt. medical college Jammu were included in the study for a period of one year from October 2014 to September 2015. It was a prospective study. In the present study, out of 70 cases, majority of the patients 57 (81.43%) belonged to haemophilia A (FVIII deficiency) followed by 12 (17.14%) patients of haemophilia B (FIX deficiency) and 1(1.43%) patient showed deficiency of both (FVIII/IX deficiency).Majority of the patients were of severe haemophilia, followed by moderate and mild haemophilia . Out of 70 cases, 58.58% were associated with positive family history of haemophilia .Majority of the patients (31.43%) were in the age range of 11 to 20 years followed by 25.71% patients seen in age group 21-30 years. In most of the haemophilia patients (52.86%), age of manifestation of symptoms was seen in 1-5years of age group. Most common clinical presentation was found to be hemarthosis (68.57%) followed by muscle and subcutaneous hematomas (45.71%). knee joint (61.43%) was the predominantly effected joint in haemophilia followed by elbow joint (41.43%). Haemophilias are distributed worldwide and have heterogenous presentation depending upon disease severity. Knowledge of the spectrum of presentation of haemophilia in the local population helps in early diagnosis and management planning.