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Semin Thromb Hemost 2015; 41(03): 323-329
DOI: 10.1055/s-0034-1395350
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Factor XIII Deficiency in Iran: A Comprehensive Review of the Literature

Akbar Dorgalaleh
1   Department of Hematology, Allied Medical School, Tehran University of Medical Sciences, Tehran, Iran
,
Majid Naderi
2   Department of Pediatrics Hematology & Oncology, Ali Ebn-e Abitaleb Hospital Research Center For Children And Adolescents Health [RCCAH], Zahedan University of Medical Sciences, Zahedan, Iran
,
Maryam Sadat Hosseini
1   Department of Hematology, Allied Medical School, Tehran University of Medical Sciences, Tehran, Iran
,
Shaban Alizadeh
1   Department of Hematology, Allied Medical School, Tehran University of Medical Sciences, Tehran, Iran
,
Soudabeh Hosseini
3   Departments of Hematology, Allied Medical School, Iran University of Medical Sciences, Tehran, Iran
,
Shadi Tabibian
1   Department of Hematology, Allied Medical School, Tehran University of Medical Sciences, Tehran, Iran
,
Peyman Eshghi
4   Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran
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Publikationsdatum:
23. Januar 2015 (online)

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Abstract

Factor XIII deficiency (FXIIID) is a rare bleeding disorder with an estimated prevalence of 1 in 2-million population worldwide. In Iran, a Middle Eastern country with a high rate of consanguineous marriages, there are approximately 473 patients afflicted with FXIIID. An approximately 12-fold higher prevalence of FXIIID is estimated in Iran in comparison with overall worldwide frequency. In this study, we have undertaken a comprehensive review on different aspects of FXIIID in the Iranian population. The distribution of this disease in different regions of Iran reveals that Sistan and Baluchestan Province has not only the highest number of patients with FXIIID in Iran but the highest global incidence of this condition. Among Iranian patients, umbilical cord bleeding, hematoma, and prolonged wound bleeding are the most frequent clinical manifestations. There are several disease causing mutations in Iranian patients with FXIIID, with Trp187Arg being the most common mutation in FXIIID in Iran. Traditionally, the management of FXIIID in Iran was only based on administration of fresh frozen plasma or cryoprecipitate, until 2009 when FXIII concentrate became available for patient management. Various studies have evaluated the efficacy and safety of prophylactic regimens in different situations with valuable findings. Although the focus of this study is on Iran, it offers considerable insight into FXIIID, which can be applied more extensively to improve the management and quality of life in all affected patients.

Keywords

factor XIII deficiency - Iran - molecular spectrum - clinical manifestations
 

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