Factor V inhibitor associated with Sjögren's syndrome
| Autor: | Takayoshi Kusano, Takatoshi Koyama, Takako Saito, Shinsaku Hirosawa |
|---|---|
| Rok vydání: | 2008 |
| Předmět: |
Male
medicine.medical_specialty medicine.drug_class Immunoblotting Internal medicine medicine Humans Blood Coagulation Aged Autoimmune disease Prothrombin time medicine.diagnostic_test biology business.industry Anticoagulant Factor V Autoantibody Hematology medicine.disease Sjogren's Syndrome Endocrinology Immunology Prothrombin Time biology.protein Immunoglobulin Light Chains Partial Thromboplastin Time Antibody business Protein C Partial thromboplastin time medicine.drug |
| Zdroj: | British Journal of Haematology. 89:893-896 |
| ISSN: | 0007-1048 |
| Popis: | We report an unusual case of a 74-year-old male who developed a serum autoantibody reactive with human coagulation factor V (FV) in an activated form, as demonstrated by coagulation studies and immunoblotting analysis. Despite marked prolongation of a prothrombin time and an activated partial thromboplastin time in this patient, the inhibitor was not associated with clinical bleeding but with multiple cerebral infarctions. The patient had suffered from Sjogren's syndrome with polyclonal hypergamma-globulinaemia. The patient's purified IgG, an immediately acting inhibitor to FV, reacted with a light chain of thrombin-activated FV (FVa) and inhibited the procoagulant activity of FVa without affecting the cleavage of FVa by activated protein C. The FV inhibitor may arise from activation of FV with consequent exposure of neoantigen during the activation of coagulation cascade in the patient with an autoimmune disorder for the background. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Plný text