Management of rare acquired bleeding disorders

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish
Pages (from-to)80-87
Number of pages8
JournalHematology (United States)
Volume2019
Issue number1
DOIs
Publication statusPublished - Dec 6 2019

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Autoantibodies
Hemorrhage
Blood Coagulation Factors
Early Diagnosis
Immunosuppressive Agents
Fibrinogen
Therapeutics
Pharmaceutical Preparations
factor VIII, von Willebrand factor drug combination
Acquired Factor 8 deficiency

ASJC Scopus subject areas

  • Hematology

Cite this

Management of rare acquired bleeding disorders. / Menegatti, Marzia; Biguzzi, Eugenia; Peyvandi, Flora.

In: Hematology (United States), Vol. 2019, No. 1, 06.12.2019, p. 80-87.

Research output: Contribution to journalArticle

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