General principles and modalities of management of thrombosis in congenital dysfibrinogenemia: case 3
| Investigation of thrombosis • Screening of CD should be considered as a second- or third-line investigation in selected patients and families after having ruled out the more common causes of thrombophilia. |
| Treatment of venous thrombosis • For patients with type 3A dysfibrinogenemia, we adopt the same recommendations as for the general population, favoring a limited duration of anticoagulation (ie, 3-6 mo). • For patients with type 3B dysfibrinogenemia, we propose a long-term anticoagulation. • Anticoagulation with a direct anticoagulant is our first choice. Low-molecular-weight heparin is the second choice. A vitamin K antagonist may be considered if the baseline PT is not prolonged. |
| Thromboprophylaxis • For patients with type 3A dysfibrinogenemia we adopt the same recommendations as for the general population, favoring a mechanical thromboprophylaxis whenever possible. • For patients with type 3B dysfibrinogenemia, we adopt the same recommendations as for the general population favoring a pharmacological thromboprophylaxis, whenever possible. |
| Investigation of thrombosis • Screening of CD should be considered as a second- or third-line investigation in selected patients and families after having ruled out the more common causes of thrombophilia. |
| Treatment of venous thrombosis • For patients with type 3A dysfibrinogenemia, we adopt the same recommendations as for the general population, favoring a limited duration of anticoagulation (ie, 3-6 mo). • For patients with type 3B dysfibrinogenemia, we propose a long-term anticoagulation. • Anticoagulation with a direct anticoagulant is our first choice. Low-molecular-weight heparin is the second choice. A vitamin K antagonist may be considered if the baseline PT is not prolonged. |
| Thromboprophylaxis • For patients with type 3A dysfibrinogenemia we adopt the same recommendations as for the general population, favoring a mechanical thromboprophylaxis whenever possible. • For patients with type 3B dysfibrinogenemia, we adopt the same recommendations as for the general population favoring a pharmacological thromboprophylaxis, whenever possible. |