From the Guidelines
Disseminated Intravascular Coagulation (DIC) and afibrinogenemia are distinct coagulation disorders with different causes and presentations, and the most recent evidence suggests that their management should prioritize treating the underlying cause and providing supportive care with blood products for DIC, while afibrinogenemia is treated with fibrinogen replacement therapy 1.
Key Differences
- DIC is an acquired condition characterized by widespread, inappropriate activation of the clotting system throughout the body, resulting in simultaneous excessive clotting and bleeding, whereas afibrinogenemia is a rare inherited disorder characterized by the absence or extremely low levels of fibrinogen in the blood.
- DIC occurs secondary to another underlying condition such as sepsis, trauma, malignancy, or obstetric complications, whereas afibrinogenemia is caused by genetic mutations affecting fibrinogen production rather than consumption.
- Laboratory findings in DIC typically show prolonged prothrombin time and partial thromboplastin time, decreased platelets and fibrinogen, and elevated D-dimer levels, whereas in afibrinogenemia, laboratory findings typically show low or undetectable fibrinogen levels.
Treatment Approaches
- DIC management focuses on treating the underlying cause and providing supportive care with blood products, such as platelet transfusions and fresh frozen plasma, as suggested by the International Society on Thrombosis and Haemostasis (ISTH) 1.
- Afibrinogenemia is treated with fibrinogen replacement therapy, typically using cryoprecipitate or fibrinogen concentrates to prevent or control bleeding episodes, with a recommended threshold of >100 mg/dl to trigger fibrinogen replacement in the setting of bleeding 1.
Recent Evidence
- A recent study published in 2025 highlights the importance of distinguishing between DIC and cirrhotic coagulopathy, and emphasizes the need for further research on the role of fibrinogen in the pathophysiology of bleeding in patients with cirrhosis 1.
- Another study published in 2022 suggests that fibrinogen levels <100 mg/dl are associated with spontaneous and procedure-related bleeding in patients with cirrhosis, and recommends a fibrinogen threshold of >120 mg/dl to trigger fibrinogen replacement in the setting of bleeding 1.
From the Research
Difference between DIC and Afibrinogenemia
- Disseminated Intravascular Coagulation (DIC) is a systemic pathophysiologic process characterized by the activation of coagulation, consumption of platelets and coagulation factors, and formation of microvascular fibrin thrombi, leading to multiorgan dysfunction syndrome 2.
- Afibrinogenemia, on the other hand, is a rare bleeding disorder characterized by the complete absence of fibrinogen, a critical coagulation factor, in the blood.
- The key difference between DIC and afibrinogenemia is that DIC is a consumptive coagulopathy, where coagulation factors and platelets are consumed due to widespread clotting, whereas afibrinogenemia is a deficiency of a specific coagulation factor, fibrinogen.
- In DIC, the coagulation system is activated, leading to the formation of thrombi, whereas in afibrinogenemia, the coagulation system is impaired due to the lack of fibrinogen, leading to bleeding complications.
- Treatment of DIC involves addressing the underlying cause, replacing coagulation factors and platelets, and managing bleeding and thrombotic complications 3, 4, whereas treatment of afibrinogenemia typically involves replacement of fibrinogen using cryoprecipitate or fibrinogen concentrate 5.
Clinical Presentation
- DIC can present with a range of clinical manifestations, including bleeding, thrombosis, and organ dysfunction, depending on the underlying cause and severity of the condition 2.
- Afibrinogenemia typically presents with bleeding complications, such as easy bruising, bleeding gums, and heavy menstrual bleeding in women.
- Laboratory tests, such as coagulation studies and fibrinogen levels, can help differentiate between DIC and afibrinogenemia 4, 6.
Management
- Management of DIC involves a multidisciplinary approach, including treatment of the underlying cause, replacement of coagulation factors and platelets, and management of bleeding and thrombotic complications 3, 4.
- Management of afibrinogenemia typically involves replacement of fibrinogen using cryoprecipitate or fibrinogen concentrate, and may require ongoing therapy to prevent bleeding complications 5.