Hemorrhagic, thrombotic and obstetric complications of congenital dysfibrinogenemia in a previously asymptomatic woman

Peterson, Wynn; Liederman, Zachary; Baker, Jennifer M.; Martin, Rachel; Berger, Howard; Meffe, Filomena; Teitel, Jerry; Sholzberg, Michelle

doi:10.1016/j.thromres.2020.08.028

Cited by 7 publications

(4 citation statements)

References 11 publications

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“…The phenomenon of actual lower than expected fibrinogen activity has been found in previous studies according to subtle changes in fibrinogen activity. [ 12 , 13 ] The observation has supported that dysfunctional fibrinogen may interfere with the function of replacing fibrinogen. [ 6 ] As this case shows, during the first course, changes in the level of fibrinogen and FDP would suggest hyperfibrinolysis given the management of low fibrinogen.…”

Section: Discussionmentioning

confidence: 99%

“…The relationship between CD and complications following TKA is less clearly defined, although in many pregnancy cases, there is still no unified application of the aforementioned drugs. [ 12 ] Furthermore, the coagulation balance in asymptomatic CD patients should be carefully considered for treatment with FC, TXA, and LMWH.…”

Section: Discussionmentioning

confidence: 99%

“…[4,11] Since asymptomatic pregnant women are more likely to be diagnosed because of indispensable pregnancy tests, there are recommendations for managing pregnancy in CD patients. [12] However in a selective operation, most therapeutic schedules refer to CH patients.…”

Section: Discussionmentioning

confidence: 99%

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A low-dose therapy of fibrinogen supplement during perioperative period of total knee arthroplasty in an asymptomatic man with congenital dysfibrinogenemia: A case report

Meng

Zhang

et al. 2022

Medicine

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Rationale: Congenital dysfibrinogenemia (CD) is a rare coagulation system disease that is often treated without unified management. Individualized treatment thereof presents clinicians with great challenges.Patient concerns: A patient who was about to undergo total knee arthroplasty was found to have CD.Diagnoses: Coagulation screening revealed low fibrinogen, prolonged thrombin time, minor prolonged prothrombin time, and normal activated partial thromboplastin time were detected during admission, but no abnormal personal and family history findings were observed. Therefore, CD and hypofibrinogenemia were suspected. The gene sequencing confirmed the diagnosis of CD. Interventions:The patient received plenty and low level of fibrinogen concentrate during 2 perioperative periods, respectively.Outcomes: Successful clinical outcomes were obtained using different treatment strategies.Lessons: In contrast to prior case reports, this case illustrates the feasibility of low dosing of fibrinogen supplements within an asymptomatic patient in a selective operation. Changes in the level of fibrinogen and fibrin degradation product are of great importance for individualized treatment after supplementation.Abbreviations: CD = congenital dysfibrinogenemia, CH = congenital hypofibrinogenemia, DVT = deep vein thrombosis, FC = fibrinogen concentrate, LMWH = low molecular weight heparin, TKA = total knee arthroplasty, TXA = tranexamic acid.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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A low-dose therapy of fibrinogen supplement during perioperative period of total knee arthroplasty in an asymptomatic man with congenital dysfibrinogenemia: A case report

Meng

Zhang

et al. 2022

Medicine

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“…Pregnancy is a high-risk clinical situation in women who have CD. 77 Miscarriage, 78 stillbirth, 13 placenta abruptio, 79 intrauterine growth restriction, 80 PPH, 81 and thrombosis 82 have been reported, although most pregnancies progress smoothly. In a cohort of 111 pregnancies, the miscarriage rate was 19.8%, 13 even though, in the subgroup of women with a thrombosis-related mutation, the rate rose significantly to about 40%.…”

Section: Discussion Of Casementioning

confidence: 99%

How I treat dysfibrinogenemia

Casini

Moerloose

2021

Blood

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Congenital dysfibrinogenemia (CD) is caused by structural changes in fibrinogen that modify its function. Diagnosis is based on discrepancy between decreased fibrinogen activity and normal fibrinogen antigen levels and is confirmed by genetic testing. CD results from monoallelic mutations in fibrinogen genes leading to clinically heterogenous disorders. Most patients with CD are asymptomatic at time of diagnosis but the clinical course may be complicated by a tendency to bleeding and/or thrombosis. Patients with a thrombotic-related fibrinogen variant are particularly at risk and in such patients long-term anticoagulation should be considered. Management of surgery and pregnancy raise important and difficult issues. The mainstay of CD treatment remains fibrinogen supplementation. Antifibrinolytic agents are part of the treatment in some specific clinical settings. In this article, we discuss five clinical scenarios to highlight common clinical challenges. We detail our approach to establish a diagnosis of CD and discuss strategies for the management of bleeding, thrombosis, surgery and pregnancy.

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Congenital Fibrinogen Disorders, Diagnosis, and Management

Casini

2023

Congenital Bleeding Disorders

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Hemorrhagic, thrombotic and obstetric complications of congenital dysfibrinogenemia in a previously asymptomatic woman

Cited by 7 publications

References 11 publications

A low-dose therapy of fibrinogen supplement during perioperative period of total knee arthroplasty in an asymptomatic man with congenital dysfibrinogenemia: A case report

A low-dose therapy of fibrinogen supplement during perioperative period of total knee arthroplasty in an asymptomatic man with congenital dysfibrinogenemia: A case report

How I treat dysfibrinogenemia

Congenital Fibrinogen Disorders, Diagnosis, and Management

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