Factor V inhibitor associated with Sjögren's syndrome |
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Authors: | Takatoshi Koyama Takako Saito Takayoshi Kusano SHINSAKU Hirosawa |
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Institution: | The First Department of Internal Medicine;School of Allied Health Sciences;Department of Laboratory Medicine, Tokyo Medical and Dental University, Tokyo, Japan |
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Abstract: | 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 Sjögren'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. |
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Keywords: | factor V inhibitor activated factor V light chain Sjögren's syndrome hypergammaglobulinaemia activated protein C |
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