Coagulation Factor Autoantibodies

Massimo Cugno, Alberto Tedeschi

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

The coagulation system consists of a cascade of proteolytic reactions in which factors are activated, leading to the formation of a fibrin clot that can stop bleeding from a damaged vessel. In some conditions, autoantibodies may develop against coagulation factors, altering their function or promoting their rapid clearance. Coagulation factor autoantibodies are rarely encountered in noncongenitally deficient patients; however, when present, they are in association with autoimmune diseases, lymphoid malignancies, and pregnancy, as well as advanced age. When a patient presents with bleeding symptoms without any prior bleeding diathesis, the possible development of coagulation factor autoantibodies should be considered. Acquired factor VIII deficiency is the most common disorder and is characterized by hemorrhages involving soft tissues, muscle, and skin; hemarthroses are less frequent than in the inherited form. Acquired deficiencies of von Willebrand factor and factor XIII due to autoantibodies are emerging conditions. Autoantibodies to the other coagulation factors may be associated with a wide spectrum of clinical manifestations ranging from minimal or no bleeding to life-threatening conditions. The diagnostic approach starts with global coagulation tests: prothrombin time (PT) and activated thromboplastin time (aPTT). In case of prolonged times, mixing studies (typically using normal plasma in a 1:1 proportion) are performed. Specific factor assays, assessment of lupus anticoagulant, and eventually enzyme immunoassays for specific antifactor antibodies can complete the evaluation. A prompt diagnosis of a specific coagulation factor inhibitor is crucial for starting an appropriate treatment aimed at the replacement or overcoming of the deficient factor, in case of bleeding, and at the elimination of the autoantibody as well as the suppression of its production.

Original languageEnglish
Title of host publicationAutoantibodies: Third Edition
PublisherElsevier B.V.
Pages499-509
Number of pages11
ISBN (Print)9780444563781
DOIs
Publication statusPublished - Dec 2013

Keywords

  • Acquired hemophilia
  • Acquired inhibitors
  • Autoantibodies
  • Coagulation
  • Factor IX
  • Factor VIII
  • Hemostatic agents
  • Von Willebrand factor

ASJC Scopus subject areas

  • Immunology and Microbiology(all)

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