Challenges Regarding the Management of Gynecological and Obstetric Complications in Women with Inherited Factor XIII Deficiency

Abstract

The medical care of patients with inherited bleeding disorders requires a greater understanding and attention, especially in women who can be exposed to specific hemorrhagic complications such as menorrhagia or complications during pregnancies and deliveries. These potential complications have to be taken into account, and specific procedures or treatment regimens must be provided. Among the general population with bleeding disorders, hemophilia A and B along with von Willebrand disease represent about 95% to 97% of cases. The remaining disorders, called rare bleeding disorders (RBDs), are due to fibrinogen, or factor II, V, VII, X, XI, or XIII (FXIII) deficiencies [1]. Among these RBDs, the congenital FXIII deficiency (FXIIID) is a very rare life-threatening autosomal recessive bleeding disorder and also a cause of recurrent miscarriages. Due to the rarity of these diseases and the consequent absence of randomized controlled studies investigating treatment, recommendations for their management are mainly based on expert consensus rather than on evidence-based guidelines [2]. Since 2012, the European Network and the United Kingdom Haemophilia Centre Doctors’ Organization (UKHCDO) have published recommendations for the management of RBDs, including the management of FXIIID [3-5]. In parallel, other authors published specific recommendations regarding FXIIID treatment [6,7].