Inherited coagulation disorders in southern Iran

Area of Science:

• Hematology
• Epidemiology
• Public Health

Background:

• Hemophilia and allied bleeding disorders represent a significant public health concern.
• Understanding the demographics and clinical manifestations of these disorders is crucial for effective management.
• Previous data on bleeding disorders in southern Iran was limited.

Purpose of the Study:

• To conduct a comprehensive survey of patients with hemophilia and related bleeding disorders in southern Iran.
• To collect data on patient demographics, clinical signs, and symptoms.
• To assess the prevalence of transfusion-transmitted infections.

Main Methods:

• A survey was conducted in April 2002 at the Shiraz Hemophilia Society and its treatment center.
• Data was collected on 367 patients diagnosed with coagulation disorders.
• Patient records were analyzed for demographic information, symptoms, and infection status.

Main Results:

• Hemophilia A (Factor VIII deficiency) was the most prevalent (n=271), followed by Hemophilia B (FIX deficiency) (n=39) and von Willebrand disease (n=24).
• Rare coagulation disorders affected 33 patients, including Factor X, VII, XIII, XI deficiencies, and combined factor deficiencies.
• The overall prevalence of coagulation disorders was 6.64 per 100,000 inhabitants. Common symptoms included hemarthrosis, hematomas, and epistaxis.
• No patients were HIV positive; 15% were Hepatitis C virus positive, and 0.7% were Hepatitis B positive.

Conclusions:

• Hemophilia A is the predominant bleeding disorder in this southern Iranian population.
• The prevalence of transfusion-transmitted infections, particularly HIV, is low compared to other regions.
• This data provides valuable insights for regional healthcare planning and hemophilia management.