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Thrombosis in rare bleeding disorders
1National Haemophilia Center, Banco Metropolitano de Sangre, Caracas, Venezuela. arletteruizsaez@gmail.com
Hematology (Amsterdam, Netherlands)
|April 18, 2012
Summary
Inherited bleeding disorders can paradoxically lead to thrombosis. Management requires individualized replacement therapy and consideration of antithrombotic prophylaxis, especially in patients with a history of clotting events.
Area of Science:
- Hematology
- Thrombosis and Hemostasis
Background:
- Inherited coagulation factor deficiencies typically cause bleeding, but paradoxically, thrombotic events are reported.
- Conditions include Hemophilias A and B, von Willebrand disease, and deficiencies of fibrinogen, prothrombin (FII), FV, FVII, FX, FXI, FXIII, and combined factors.
Purpose of the Study:
- To review the paradoxical occurrence of thrombosis in patients with inherited bleeding disorders.
- To discuss the multifactorial pathogenesis of thrombosis in hemophilia and other rare bleeding disorders.
- To provide recommendations for individualized management and thrombophilia screening.
Main Methods:
- Literature review of thrombotic events in inherited coagulation factor deficiencies.
- Analysis of risk factors and pathogenetic mechanisms for thrombosis.
- Synthesis of current recommendations for patient management.
Main Results:
- Thrombosis in hemophilia is linked to central venous catheters, intensive replacement therapy, bypassing agents, and prothrombotic risk factors.
- Thrombotic phenomena are described in afibrinogenemia, FXI, and FVII deficiencies, occurring even in young patients.
- Events can be spontaneous or associated with risk factors.
Conclusions:
- Individualized replacement therapy is crucial, considering bleeding and thrombosis history, family history, and factor levels.
- Antithrombotic prophylaxis may be considered for mild, asymptomatic deficiencies.
- Thrombophilia screening and cardiovascular risk factor control are recommended for affected patients.
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