Three successful deliveries involving a woman with congenital afibrinogenaemia – conventional fibrinogen concentrate infusion vs. ‘as required’ fibrinogen concentrate infusion based on changes in fibrinogen clearance –

Oda, Tomoaki; Itoh, Hiroaki; Kawai, Kenta; Oda-Kishimoto, A.; Kobayashi, Takao; Doi, Takahiro; Uchida, Toshiyuki; Kanayama, Naohiro

doi:10.1111/hae.13054

Cited by 9 publications

(9 citation statements)

References 8 publications

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“…After increasing the fibrinogen substitution, the haematoma resolved and course of pregnancy was uncomplicated, with median fibrinogen levels maintained around 0.60 g/L . Another afibrinogenaemic woman had three uneventful pregnancies under a fibrinogen concentrate prophylaxis targeting about 1 g/L and 1.5‐2 g/L in the second part of the pregnancy . One third of hypofibrinogenaemic women (24.7%) received a fibrinogen replacement as prophylaxis, mainly because of past history of obstetrical complications.…”

Section: Resultsmentioning

confidence: 99%

Obstetrical and postpartum complications in women with hereditary fibrinogen disorders: A systematic literature review

et al. 2019

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Introduction: Hereditary fibrinogen disorders (HFD) are rare quantitative or qualitative fibrinogen anomalies, including afibrinogenaemia (A), hypofibrinogenaemia (H), dysfibrinogenaemia (D) and hypodysfibrinogenaemia (HD). As fibrinogen plays an essential role in pregnancy, we addressed the issue of obstetrical and postpartum complications in women with HFD. Methods: A systematic literature review, restricted to English manuscripts, was conducted according to the PRISMA guidelines. We searched through the MEDLINE database for English articles, published from January 1985 until November 2018, focusing on pregnancy in A, H, D and HD. A total of 198 articles were identified, 15 articles were added from other sources. Overall, 213 articles were screened and 54 were included in the final analysis.Results: A total of 188 pregnancies from 70 women were analysed. About half of pregnancies resulted in miscarriage; more specifically in 15 (42.9%), 36 (46.8%), 27 (42.9%) and 4 (30.8%) of A, H, D and HD patients, respectively. Preterm complications were also frequent (33.5%). Metrorrhagia, mainly in the first trimester, was observed in 21.7% of the pregnancies. Placenta abruption was reported in 5 (14.3%), 4 (5.2%), 5 (7.9%) and 1 (7.7%) of A, H, D and HD, respectively. A total of 24 (12.7%) deliveries were complicated by postpartum thrombotic events (3.2%) or postpartum haemorrhage (9.6%). A fibrinogen replacement therapy was introduced in 30% of pregnancies, as prophylaxis (81.1%) or on demand (18.9%). Conclusion:These results suggest that women with HFD are at high risk of obstetrical and postpartum complications. Prospective international registries may allow to identify more precisely the incidence of obstetrical and postpartum adverse outcomes and their management. K E Y W O R D S afibrinogenaemia, dysfibrinogenaemia, hypofibrinogenaemia, miscarriage, postpartum haemorrhage, pregnancy

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Section: Resultsmentioning

confidence: 99%

Obstetrical and postpartum complications in women with hereditary fibrinogen disorders: A systematic literature review

et al. 2019

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“…Overall, as already reported by the analysis of more than 27 years of pharmacovigilance data on Haemocomplettan P ® , fibrinogen concentrate shows a promising safety profile. 40 Similarly, after an intracranial bleeding, a frequent and leading cause of morbidity and mortality in patients with afibrinogenemia, data are lacking to indicate a role for primary prophylaxis. issues are still unanswered.…”

Section: Safe T Ymentioning

confidence: 99%

Fibrinogen concentrates in hereditary fibrinogen disorders: Past, present and future

Casini

Moerloose²

2019

Haemophilia

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Hereditary fibrinogen disorders (HFD) are rare coagulation disorders resulting from mono-or bi-allelic mutations in fibrinogen genes. 1According to the levels of fibrinogen activity and antigen, HFD are classified into quantitative (afibrinogenemia and hypofibrinogenemia) or qualitative (dysfibrinogenemia and hypodysfibrinogenemia) disorders. 2 The spectrum of symptoms is broad and heterogeneous among patients with HFD, even if bleeding is the most common complication of all HFD sub-types. 3 The bleeding phenotype is mostly correlated to the fibrinogen level, 4 ranging from life-threatening haemorrhage to postsurgical bleeding. In afibrinogenemia, the majority of patients suffer from severe bleeding such as umbilical cord bleeds, muscle haematomas, haemarthroses and bleeding in the central nervous system. 5 Paradoxically, thrombotic events can occur in patients with afibrinogenemia, even in the absence of fibrinogen administration. 6 In hypofibrinogenemia, spontaneous bleeding is less frequent and complications are mainly associated with trauma or surgery. 7 In dysfibrinogenemia, the cumulative incidence of major bleeding has been estimated to be about 19% at 50 years of age. 8 However, some dysfibrinogen variants are strongly associated with an increased risk of thrombosis. 9 In all types of HFD, surgery and pregnancy are high-risk bleeding situations.As for most coagulation factor deficiencies, in HFD, the bleeding prevention and management is based on replacement of the lacking protein. Three sources of fibrinogen are currently available: fresh frozen plasma, cryoprecipitate and lyophilized fibrinogen concentrate. 10 The latter is the best option, although still not widely available. It confers several advantages compared to fresh frozen plasma and cryoprecipitate: (a) it represents a safer therapeutic option due AbstractHereditary fibrinogen disorders (HFD) are rare coagulation disorders. Even if the spectrum of symptoms is broad depending on the sub-type, bleeding is the most common complication. Of the available sources of fibrinogen replacement, fibrinogen concentrate provides a safer and more effective option to treat and prevent bleeding.Recent clinical trials on established and new fibrinogen concentrates have increased our knowledge on the clinical pharmacology of these products, pointing out possible age and weight differences for dose adjustment. The efficacy of fibrinogen infusions has been demonstrated, especially for the management of acute bleeding with an excellent response based on investigator rating. The target fibrinogen levels in the setting of both minor and major surgeries have been better specified. The safety has been confirmed with a low number of adverse events but there still remains concern over possible thrombotic risks. Pharmacological, clinical aspects and future perspectives on the utilization of fibrinogen concentrates in the treatment and prevention of bleeding in patients with HFD are reviewed.

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“…These cases illustrate the difficulties faced when treating women with congenital afibrinogenemia, who experience an increased risk of both hemorrhage and thrombosis during pregnancy and in the peripartum period. Large‐scale studies on this topic do not exist, but most guidelines, including the Dutch national guidelines, currently advise the maintenance of fibrinogen levels at ≥0.6 g/L during pregnancy to prevent fetal loss; this level is based on expert opinion and case reports . Interestingly, although the target level was reached during the second pregnancy, bleeding complications still occurred.…”

Section: Discussionmentioning

confidence: 99%

“…Black = patient 1, white = patient 2, *in response to this, target fibrinogen levels were raised to ≥1.0 g/l for both patients, (B) Family tree. Grey = unconfirmed carrier 13 Note: Patient numbers correspond to the associated number in the family tree in Figure 1B.…”

Section: Discussionmentioning

confidence: 99%

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Pregnancy outcome in afibrinogenemia: Are we giving enough fibrinogen concentrate? A case series

Saes

Gorkom

Coppens

et al. 2020

Research and Practice in Thrombosis and Haemostasis

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Congenital afibrinogenemia is a rare autosomal recessive disorder associated with an increased risk of hemorrhage, thrombosis, and obstetric complications. This case series of 4 pregnancies in 2 related patients seeks to address the key clinical question of the necessary doses of fibrinogen concentrate during pregnancy and puerperium. One pregnancy without the prophylactic use of fibrinogen concentrate resulted in spontaneous abortion. The second pregnancy was complicated by a subchorionic hematoma despite the prophylactic administration of fibrinogen concentrate to maintain the plasma trough levels at ≥0.6 g/L. Labor was complicated by postpartum hemorrhage with a blood loss volume of 1480 cc. Two weeks later, the patient presented with postpartum thrombosis. The other 2 pregnancies were uncomplicated with fibrinogen trough levels ≥1.0 g/L during pregnancy and ≥1.5 g/L during labor. These cases illustrate that during pregnancy, patients may benefit from fibrinogen trough levels ≥1.0 g/L. In addition, the increased risk of postpartum thrombosis with prolonged fibrinogen supplementation warrants personalized postpartum advice that is guided by postpartum blood loss.

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Three successful deliveries involving a woman with congenital afibrinogenaemia – conventional fibrinogen concentrate infusion vs. ‘as required’ fibrinogen concentrate infusion based on changes in fibrinogen clearance –

Abstract: Three successful deliveries involving a woman with congenital afibrinogenaemiaconventional fibrinogen concentrate infusion vs. 'as required' fibrinogen concentrate infusion based on changes in fibrinogen clearance -

Cited by 9 publications

References 8 publications

Obstetrical and postpartum complications in women with hereditary fibrinogen disorders: A systematic literature review

Obstetrical and postpartum complications in women with hereditary fibrinogen disorders: A systematic literature review

Fibrinogen concentrates in hereditary fibrinogen disorders: Past, present and future

Pregnancy outcome in afibrinogenemia: Are we giving enough fibrinogen concentrate? A case series

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