Abstract Number: PB0730
Meeting: ISTH 2020 Congress
Background: Congenital fibrinogen disorders are rare and challenging disorders in pregnancy with no firm treatment guidelines and only case reports are published. The clinical course of these disorders varies from no manifestation, to bleeding or thrombotic events. Pregnant women with congenital fibrinogen disorders suffer from recurrent pregnancy loss. Patients were reffered to our centre after repeating miscarriages, unsuccesfull IVFs or through their hypo/dysfibrinogenemia family history.
Aims: We present 8 succesfull pregnancies and their management in 7 patients.
Methods: Seven patients and 8 pregnancies were treated between 2008-2019: 6x dysfibrinogenemia and 1x hypofibrinogenemia. Average age was 32 years (22-41) at delivery. Standard laboratory before pregnancy was: Clauss fibrinogen level, fibrinogen antigen level, additional functional tests (RT, TT, fibrin polymerization, TEG) and trombofilia (1x FVL heterozygot). Every pregnancy was laboratory monitored by fibrinogen and DD levels in each trimester. In all patients was proven fibrinogen heterozygous missense mutation. Our mutation findings were correlated with international registry (Human fibrinogen database) and its clinical manifestations.
Results: When sampling this unique patient cohort, we discovered one new mutation called Fibrinogen Praha III“. The fibrinogen substitution was used as prophylaxis in 6/8 deliveries and 1 amniocenthesis. Therapeutic fibrinogen substitution was in bleeding complications. Five pregnancies were covered by LMWH in prophylactic/therapeutic dose concerning laboratory and clinical presentation. We have treated 3 serious complications in 8 succesfull pregnancies: 1 patient with pulmonary embolism in the 1th trimester despite LMWH prohylaxis, 2. patient with hypofibrinogenemia presented with severe bleeding in the 2nd and preeklampsia in the 3rd trimester. Fibrinogen treatment led to no trombotic complications and vice versa LMWH had no bleeding complication.
Conclusions: Treatment of patients with so rare diagnosis is recommended in specialized centre. Further follow-up of patiens, international registry collaboration and effort to establish treatment guidelines is warranted.
|Patient||Mutation||D/H||Phenotyp in register(B/T)||Previous miscarriages||Bleeding(B)||TEN (T)||Delivery|
|P5||Aa13Gly/Glu||D||Y/N||3+unsucc. IVF||1th trimester||0||2011|
[Pregnant patients with congenital fibrinogen disorders – dys(D) or hypo(H)fibrinogenemia]
To cite this abstract in AMA style:Pohlreichová V, Čapová I, Geierová V, Loužil J, Ceznerová E, Kotlín R, Hrachovinová I, Salaj P. Eight Succesfull Pregnancies in Patients with Congenital Fibrinogen Disorders – Single Centre Study [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/eight-succesfull-pregnancies-in-patients-with-congenital-fibrinogen-disorders-single-centre-study/. Accessed May 6, 2021.
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