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[Immune coagulation disorders (excluding antiphospholipid syndrome].
1Department of Laboratory Medicine, Tokyo Medical University, Shinjuku-ku, Tokyo 160-0023
Rinsho Byori. the Japanese Journal of Clinical Pathology
|January 5, 2002
Summary
Patients undergoing factor replacement therapy can develop antibodies to clotting factors, particularly factor VIII inhibitors in hemophilia A. Genetic mutations and underlying autoimmune disorders are associated with inhibitor development.
Area of Science:
- Hematology
- Immunology
- Genetics
Context:
- Acquired inhibitors to coagulation factors are rare but can occur in individuals without prior bleeding disorders.
- Factor VIII inhibitors are the most common type, but antibodies can target any coagulation factor.
- Replacement therapy in severe hemophilia A carries a significant risk of alloantibody development.
Purpose:
- To investigate the incidence and characteristics of acquired inhibitors against coagulation factors.
- To identify potential genetic and underlying disease associations with inhibitor development.
- To describe cases of acquired hemophilia and acquired von Willebrand syndrome.
Summary:
- The study observed 12 hemophilia A patients with factor VIII inhibitors and 3 hemophilia B patients with factor VIII/IX inhibitors.
- Nine patients developed acquired hemophilia (autoantibody to factor VIII), and 4 had acquired von Willebrand syndrome.
- Genetic mutations (inversions, deletions, missense) were found in some factor VIII inhibitor patients, while others had autoimmune disorders.
Impact:
- Highlights the risk of alloantibody formation during factor replacement therapy.
- Underscores the importance of investigating underlying conditions in acquired inhibitor cases.
- Provides insights into the genetic and etiological factors contributing to acquired bleeding disorders.