Clinicohematological Study of Hemophilia Patients in Bhopal

The X-linked inherited coagulation disorders, hemophilia A (Factor VIII deficiency) and hemophilia B (factor IX deficiency), together with Von Willebrand disease comprise 95 to 97 percent of all the Inherited deficiencies of coagulation factors. Replacement of the deficient factor is the mainstay of treatment; it may be "on demand" or "prophylactic" to prevent hemarthrosis in severe deficiency. The prevalence of hemophilia A is 1 in 5000 and that of hemophilia B is 1 in 30,000 Clinically both the deficiencies present as a lifelong bleeding disorder with considerable morbidity due to crippling arthropathy. Most common cause of death in these patients is Intra Cerebral Hemorrhage. Hemophilia A and B can only be distinguished on the basis of specific coagulation Factor VIII or IX assays. AIM: The present study was conducted with the aim to study the clinico-hematological profile of Persons With Hemophilia(PWH), their clinical presentations, incidence of inhibitors, estimating the burden of transfusion related complications at our hemophilia care center and to compare the findings with other studies of similar nature MATERIAL & METHOD: This retrospective study was conducted in the Department of Pathology in collaboration with Hemophilia Care Centre at Gandhi Medical College, Bhopal during the period of August 2003 to July 2013. Diagnosis was made on the basis of history, physical examination and laboratory investigations such as bleeding time (BT), Prothrombin time (PT), Activated Partial Thromboplastin Time (APTT),correction studies(factor VIII and factor IX estimation whenever possible). CONCLUSION: Bleeding after injury is obvious in healthy people but difficult to decide, when it is due to bleeding disorder. Serious congenital conditions e. g. severe hemophilia becomes obvious in early childhood but may be misdiagnosed as non-accidental injury, whereas mild cases may go undetected till later years especially those who have not undergone surgery in early adult life. Presence of hemarthrosis, bruises, hematoma either spontaneous or traumatic in an otherwise normal child should warrants for investigating in the line of hemophilia. Repeated episodes early in life show severe hemophilia. Most common cause of death was found Intra Cerebral Hemorrhage (ICH).