Title: Descriptive Epidemiology and Complications of Haemophilia in Assiut, Egypt
Authors: Nabawia M. Tawfik, MBBCh, MD, Osama A. Ibrahiem, MBBCh, MD, Lobna A. Ahmed, MBBCh, MD, Rania Hafez, MBBCh, MD, Safinaz Hussein MBBCh, MD, Faisal Samir, MBBCh
DOI: https://dx.doi.org/10.18535/jmscr/v5i1.68
Abstract
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 x-linked 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.
Key Words: Haemophilia, Bleeding, Factor VIII, Factor IX, Haemoarthrosis.
