ABSTRACT
BACKGROUND Haemophilia A and haemophilia B are the commonest form of haemophilia encountered and they result from a defect in Factor VIII and Factor IX gene respectively. This hinders the process of haemostasis and predisposing haemophiliacs to spontaneous or post traumatic bleeding. We wanted to study the clinico-haematological profile of patients with haemophilia.METHODSThis observational study was conducted in Gandhi Medical College and Associated Hamidia Hospital, Bhopal, during the period of March 2017 to June 2018. After clinical evaluation, patients were subjected to a battery of coagulation tests (Bleeding Time, Prothrombin Time, Activated Partial Thromboplastin Time, Correction Studies and whenever possible, Specific Coagulation Factor Assay). The results were analysed.RESULTSDuring the study period, 100 patients of haemophilia were studied. Majority of patients were of haemophilia A (89%). Most common age group was 6 - 15 years (49%) and mean age was 19.02±12.58 years. Most common age of onset was <1 year (62%). Positive family history was present in 57% of cases. 52% patients had severe haemophilia. Most common presentation was haemarthrosis & knee joint was the most common joint involved. APTT was prolonged in all cases.CONCLUSIONSHaemophiliacs are distributed worldwide and have heterogeneous presentation depending upon disease severity. Knowledge of the spectrum of presentation of haemophilia in the population helps in early diagnosis and management planning. Promotion of regular availability of factor concentrate, establishing comprehensive care center and positive public awareness along with good haematology laboratory will help in achieving outcome comparable to that of developed countries.