Contributions
Abstract: PB1817
Type: Publication Only
Background
Hemophilia is a rare congenital disorder characterized by prolonged bleeding, either spontaneously, or after injury. In the developing world, where majority of the hemophiliacs live, awareness of this disease and its management is poorly done. It is a significant cause of morbidity and mortality and is responsible for psychological, social and economical stress to patients and their families.
Aims
The objective of this study was to evalute the frequency of Hemophilia patients diagnosed among bleeding disorders, their demographics and treatment options available for patients with hemophilia in countries with limited resources.
Methods
This is a cross sectional, observational study was carried out at the National Institute of Blood Disease Karachi, Pakistan after getting approval from the Institutional ethics committee. Diagnosed adult and pediatric hemophilia A (HA) and hemophilia B (HB) patients of various ages and severity were included from 2012-2017. Demographic and management history of hemophila patients were recorded and analyzed.
Results
A total of 149 male patients diagnosed as HA (n=107) and HB (n=42) were evaluated. Mean age of HA 13.1 ± 10.6 and for HB is 15.1 ± 11.1 years. Age at diagnosis ranged from birth to 3 years. History of consanguinity was present in 85% of cases and significant family history of bleeding in 69% of patients. Hemarthrosis and hematoma were the more frequent symptoms in these patients. Surgical history including circumcision was done in 55%patients while 4 had major surgeries (hip& femur bones fracture, extensive nasal septum, and head surgery). 29% of patients had transfusion-transmitted infections in which HCV (68%) was most prevalent followed by HBV (11%) and HIV (4%). 14 HA patients (13%) were found to have positive results for inhibitors and none in HB. Treatment included tranexamic acid, fresh frozen plasma, cryoprecipitate, cryosupernatant and factor concentrates on demand basis. Recently we have started low dose prophylaxis regimen for 15 children <10 years old with the help of World Federation of Hemophilia. We administer 250 IU vial once a week and monitor for bleeds. If no bleed, continue same dose. If 2-3 joint bleeds occur in 3 months, increase the dose to twice weekly and treat the bleed (20-30 IU/kg).
Conclusion
Hemophilia A and B are common among congenital bleeding disorders. Rate of transfusion-transmitted diseases, particularly hepatitis C infection, has gained a huge proportion. Comprehensive haemophilia care center with multidisciplinary approach needs to be established.
Session topic: 34. Bleeding disorders (congenital and acquired)
Keyword(s): Bleeding disorder, Hemophilia, Hemophilia A, Hemophilia B
Abstract: PB1817
Type: Publication Only
Background
Hemophilia is a rare congenital disorder characterized by prolonged bleeding, either spontaneously, or after injury. In the developing world, where majority of the hemophiliacs live, awareness of this disease and its management is poorly done. It is a significant cause of morbidity and mortality and is responsible for psychological, social and economical stress to patients and their families.
Aims
The objective of this study was to evalute the frequency of Hemophilia patients diagnosed among bleeding disorders, their demographics and treatment options available for patients with hemophilia in countries with limited resources.
Methods
This is a cross sectional, observational study was carried out at the National Institute of Blood Disease Karachi, Pakistan after getting approval from the Institutional ethics committee. Diagnosed adult and pediatric hemophilia A (HA) and hemophilia B (HB) patients of various ages and severity were included from 2012-2017. Demographic and management history of hemophila patients were recorded and analyzed.
Results
A total of 149 male patients diagnosed as HA (n=107) and HB (n=42) were evaluated. Mean age of HA 13.1 ± 10.6 and for HB is 15.1 ± 11.1 years. Age at diagnosis ranged from birth to 3 years. History of consanguinity was present in 85% of cases and significant family history of bleeding in 69% of patients. Hemarthrosis and hematoma were the more frequent symptoms in these patients. Surgical history including circumcision was done in 55%patients while 4 had major surgeries (hip& femur bones fracture, extensive nasal septum, and head surgery). 29% of patients had transfusion-transmitted infections in which HCV (68%) was most prevalent followed by HBV (11%) and HIV (4%). 14 HA patients (13%) were found to have positive results for inhibitors and none in HB. Treatment included tranexamic acid, fresh frozen plasma, cryoprecipitate, cryosupernatant and factor concentrates on demand basis. Recently we have started low dose prophylaxis regimen for 15 children <10 years old with the help of World Federation of Hemophilia. We administer 250 IU vial once a week and monitor for bleeds. If no bleed, continue same dose. If 2-3 joint bleeds occur in 3 months, increase the dose to twice weekly and treat the bleed (20-30 IU/kg).
Conclusion
Hemophilia A and B are common among congenital bleeding disorders. Rate of transfusion-transmitted diseases, particularly hepatitis C infection, has gained a huge proportion. Comprehensive haemophilia care center with multidisciplinary approach needs to be established.
Session topic: 34. Bleeding disorders (congenital and acquired)
Keyword(s): Bleeding disorder, Hemophilia, Hemophilia A, Hemophilia B