Bleeding Episodes Among Patients with Congenital Fibrinogen Disorders, a Study On 12 New Iranian Patients

authors:

avatar Majid Naderi 1 , * , avatar Parvin Rahamani 2 , avatar Shaban Alizadeh ORCID 3 , avatar Hengam Razavi 2 , avatar Akbar Dorgalaleh 4

Genetic Researcher Center in Non-Communicable Disease, Zahedan University of Medical sciences, Zahedan, Iran
Department of Hematology and Blood Transfusion, School of Allied Medical Sciences,Tehran University of Medical Sciences, Tehran, Iran
Associate Professor ILHA board member Tehran University of Medical Sciences, Tehran, Iran
Department of Hematology, Allied Medical School, Iran University of Medical Sciences, Tehran, Iran
Journal of Cellular & Molecular Anesthesia: Vol.3, issue 1; e150274
article type: Original Articles
DOI: https://doi.org/10.22037/jcma.v3i1.19754

how to cite: Naderi M, Rahamani P, Alizadeh S, Razavi H, Dorgalaleh A. Bleeding Episodes Among Patients with Congenital Fibrinogen Disorders, a Study On 12 New Iranian Patients. J Cell Mol Anesth. 2018;3(1):e150274. https://doi.org/10.22037/jcma.v3i1.19754.

Abstract

Background: Congenital fibrinogen disorders (CFDs) comprise about 10% of rare bleeding disorders (RBDs). CFDs are divided into two groups of quantitative (afibrinogenemia and hypofibrinogenemia) with autosomal recessive inheritance pattern, and qualitative (dysfibrinogenemia, hypodysfibrogenemia) disorders, mainly with autosomal dominant inheritance pattern. Sistan and Baluchestan Province in Iran, with its high rate of consanguineous marriages, has a high incidence of RBDs including CFD. In the current study, we report clinical manifestations of patients with CFDs.Methods: Twelve new Iranian patients from Sistan and Baluchestan Province with different types of CFDs were selected for this study. Diagnosis of CFDs was based on clinical features and familial history followed by laboratory assessment by routine and specific coagulation tests including prothrombin time (PT) and activated partial time tests (APTT), as well as FI activity assay by Clauss method.Results: Out of 12 patients, 3(25%) had afibrinogenemia, 7(58.3%) had hypofibrinogenemia while 2(16/7%) were suspected of having dysfibrinogenemia. Although umbilical cord bleeding (UCB) 9(75%) was the most common clinical presentation among the study population, this feature was not observed among patients with dysfibrinogenemia. Hematoma (100%) was the most common presentation of patients with dysfibrinogenemia.? Conclusion: Results of this study revealed that some clinical presentations are the diagnostic features of CFDs and can be used for precise and in-time diagnosis CFDs in conjunction with family history and laboratory findings.Keywords: Fibrinogen Deficiency; Congenital Afibrinogenemia; Blood Coagulation Disorder; Afibrinogenemia