An investigation of the spectrum of common and rare inherited coagulation disorders in north-eastern Iran.

Abstract

BACKGROUND:

Health care officials and legislators need accurate data on prevalence and numbers of individuals with bleeding disorders in order to plan and allot their budgets; the manufacturers of coagulation factors also need these data to estimate the amount of factors required to prevent scarcity of these products.

MATERIALS AND METHODS:

We surveyed the prevalence of haemophilia A, haemophilia B, von Willebrand's disease and rare bleeding disorders in North-Eastern Iran. The survey was done in the period from September 2009 to March 2011. Information was collected from the medical records in three major hospitals and a haemophilia centre; the patients' updated data were obtained by telephone.

RESULTS:

Overall in the current survey 552 patients with inherited coagulation disorders were identified and their medical records obtained. Of these, 429 (77.5%) had common bleeding disorders (haemophilia A, haemophilia B, von Willebrand's disease), 85 (15.6%) had rare bleeding disorders (deficiency of coagulation factors V, VII, X, XIII, I, XI, combined factor V and VIII deficiency) and 38 (6.9%) had platelet disorders.The commonest bleeding disorders were haemophilia A (n=287, 51.9%), haemophilia B (n=92, 16.6%), von Willebrand's disease (n=50, 9%), factor V deficiency (n=21, 3.8%), factor VII deficiency (n=19, 3.4%), factor X deficiency (n=2, 0.36%), combined factor V and VIII deficiency (n=28, 5.8%), factor XIII deficiency (n=11, 1.99%), factor XI deficiency (n=2, 0.4%), afibrinogenaemia (n=2, 0.36%) and platelet disorders (n=38, 6.9%).

DISCUSSION:

There is notable population of individuals with bleeding disorders in North-Eastern Iran.

• Authors: Hassan Mansouritorghabeh, Lida Manavifar, Abdollah Banihashem, Alireza Modaresi, Abbas Shirdel, Masoud Shahroudian, Ghazaleh Shoja-E-Razavi, Hamid Pousti, Habibollah Esmaily
• Journal: Blood transfusion = Trasfusione del sangue (Blood Transfus) Vol. 11 Issue 2 Pg. 233-40 (Apr 2013) ISSN: 2385-2070 [Electronic] Italy
• PMID: 23114518 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Blood Coagulation Factors

Topics

• Adolescent
• Adult
• Aged
• Aged, 80 and over
• Blood Coagulation Disorders, Inherited (epidemiology)
• Blood Coagulation Factors
• Blood Platelet Disorders (epidemiology)
• Child
• Child, Preschool
• Data Collection
• Female
• Hemophilia A (epidemiology)
• Hemophilia B (epidemiology)
• Hemorrhagic Disorders (epidemiology)
• Humans
• Infant
• Iran (epidemiology)
• Male
• Middle Aged
• Young Adult
• von Willebrand Diseases (epidemiology)