Background: Haemophilia is a group of rare congenital disorders of blood where there’s a defect in mechanism
of clotting due to deficiency in factor VIII (Haemophilia A) or factor IX (Haemophilia B). It’s inherited as xlinked
recessive disorder but 30% of patients have no family history of the disease and they usually have
spontaneous new mutation.
Aim: to describe the epidemiological situation of haemophilia in Assiut, assist the various complications and the
type of treatment.
Patients and Methods: retrospective study was conducted on 75 hemophilic patients who were attending to
Clinical Hematology Unit, Internal Medicine Department; Assiut University Hospital, Clinical Hematology Unit
of Assiut Pediatric Hospital and Hematological Clinics in Assiut Health Insurance Clinics from the period
between 2014-2016.
Results: haemophilia A represent 85.3% of the studied patients and 14.7% had haemophilia B. As regard
residency 64% of haemophilia A patients and 13.3% of haemophilia B patients live in rural areas. We found
positive consanguinity in 77.3% of hemophilic patients. Patients were classified according to the severity of
bleeding manifestations into mild haemophilia in 84.4%, moderate haemophilia in 15.6%. As regards
complications of hemophilic patients 76% had complication. The most common complications were hemarthrosis
in 26.7% patients, muscle hematoma in16% and post transfusion infections as we found Positive HCV in 5.3%. As
regard Treatment 54(72%) of all hemophilic patients were on demand treatment and 18(24%) with prophylaxis
treatment while 3(4%) with no treatment. There was statistically significant (P < 0.001) increased complications
in patients received on demand treatment compared to those received prophylaxis treatment.
Conclusions: The most common inherited bleeding disorder in our locality was haemophilia A followed by
haemophilia B. Haemophilia was more common in rural area. Increase cases with consanguineous marriages.
Hemarthrosis, hematoma and hepatitis C infection represented the main complications. Awareness, education and
genetic counseling will be needed to decrease the spread of haemophilia in our community. Factor support
(prophylaxis treatment) should be initiated for all patients as early as possible just diagnosis is confirmed to
reduce the frequency of complications. Screening of blood and blood products to reduce the risk of viral hepatitis.