Women With Congenital Hypofibrinogenemia/Afibrinogenemia: From Birth to Death

Authors: Zhang, Y., Zuo, X., and Teng, Y.

Publication: Clin Appl.Thromb Hemost.; 26, April 2020

Affiliations: Nursing Department, Baoji Maternal and Child Health Hospital, Baoji, China ; No. 986 Air Force Hospital of PLA, Xi’an, China ; Department of Obstetrics and Gynecology, the First Affiliated Hospital of Xi’an Jiaotong University, Xi’an, China ; School of Life Science and Technology, Xi’an Jiaotong University, Xi’an, China.

Abstract: Congenital fibrinogen disorders are a group of most frequent rare coagulation disorder, characterized by deficiency and/or defects in the fibrinogen molecule. Quantitative disorders include hypofibrinogenemia and afibrinogenemia. Due to their specific physiological characteristics, female patients tend to have congenital hypofibrinogenemia/afibrinogenemia, such as spontaneous recurrent abortion, menorrhagia, infertility, antepartum and postpartum hemorrhage, and so on. Current studies of congenital hypofibrinogenemia/afibrinogenemia mainly focus on different types of fibrinogen mutations, etiology/pathogenesis, and some rare case reports of the diseases. So far, there is no study available to systematically review the specific features of female patients with congenital bleeding disorders. This review aims to deal with hematological, gynecologic and obstetric issues, and relevant clinical management of congenital hypofibrinogenemia/afibrinogenemia at different life stages of female patients. We believe this review provides valuable reference for clinicians in the field of hematology, obstetrics, as well as gynecology.