Abstract
The autosomal dominantly inherited east Texas bleeding disorder is newly found in recent 10 years, It is linked to a variant in exon 13 of the coagulation factor (F)Ⅴ gene.The mutation causes abnormal splicing of FⅤ forming a FⅤ of 250 kD, named FⅤ-short. This FⅤ-short forms a complex with tissue factor pathway inhibitor(TFPI) α to inhibit the generation of thrombin and improve the concentration of TFPIα, which causes bleeding. The clinical manifestation of the disease is mostly mild bleeding, including ecchymosis, nose bleeding, bleeding after trauma, exelcymosis and surgery. The laboratory tests show prolongation of prothrombin time (PT) and activated partial thromboplastin time (APTT). The main treatment is replacement therapy at present. Key words: Hemorrhagic disorders; Factor V; East Texas bleeding disorder; Tissue factor pathway inhibitor
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