Management of rare acquired bleeding disorders
| Autor: | Flora Peyvandi, Eugenia Biguzzi, Marzia Menegatti |
|---|---|
| Rok vydání: | 2019 |
| Předmět: |
Male
Pediatrics medicine.medical_specialty Settore MED/09 - Medicina Interna Balancing the Scales: Management of Patients at Risk of Bleeding and Clotting in the Acute Care Setting Hemorrhage Disease Fibrinogen Von Willebrand factor Coagulation testing Humans Medicine Family history Autoantibodies Clotting factor biology business.industry Autoantibody Hematology Blood Coagulation Disorders Middle Aged medicine.disease Blood Coagulation Factors Bleeding diathesis biology.protein business medicine.drug |
| Zdroj: | Hematology Am Soc Hematol Educ Program |
| ISSN: | 1520-4383 1520-4391 |
| DOI: | 10.1182/hematology.2019000066 |
| Popis: | Autoantibodies toward clotting factors may develop in people suffering from autoimmune or neoplastic diseases, after drug intake or even in subjects without apparent conditions. They are more commonly directed against factor VIII (FVIII) or von Willebrand factor leading to acquired hemophilia A or acquired von Willebrand syndrome, respectively. Rarely, autoantibodies develop against other clotting factors, such as fibrinogen, FII, FV, FVII, FX, FXI, and FXIII. The clinical picture of an acquired bleeding disorder includes a wide spectrum of clinical manifestations ranging from minimal or no bleeding to life-threatening events. Patients with no previous personal or family history of bleeding may have sudden-onset hemorrhagic manifestations, sometimes fatal, especially if an early diagnosis is not made. On the other hand, some patients may not have hemorrhagic symptoms at onset, and their diagnosis can therefore be delayed. The laboratory diagnostic assessment is performed by screening coagulation tests followed by specific factor-level measurement and inhibitor-titrating assays. An early diagnosis of acquired coagulopathies is mandatory for starting the appropriate treatment aimed at both controlling the acute bleeding episode mainly using the bypassing agents, and eradicating the anticlotting factor autoantibody, using immunosuppressive treatment. Therefore, prompt intervention by an expert and a specialized center is needed for immediate recognition and treatment of the disease. |
| Databáze: | OpenAIRE |
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