FVIII measurement and TGA were performed in fresh and frozen PRP and PPP. The platelet

absence caused a significant decrease in TGA although PPP and PRP correlated well. Frozen samples gave different results in PPP, but there were no significant differences between fresh and frozen PRP. This fact enables using frozen PRP in multicenter studies with a TGA-specialized laboratory for reclassifying haemophilia severity and for pharmacokinetic studies with TGA. “
“Factor V (FV) deficiency is a rare bleeding disorder with variable bleeding manifestations that do not necessarily correlate with the level of FV activity [1]. Treatment of bleeding episodes is generally based on factor replacement through fresh frozen plasma (FFP) transfusions, as necessary, with adjunctive pharmacological this website therapy. The presence of FV inhibitor requires alternative treatment modalities that include bypassing agents and immunotolerance induction to decrease antibody titres [2]. We describe the case of a patient with FV deficiency and FV inhibitor with recurrent gastrointestinal tract (GI) bleeding from small bowel arteriovenous malformations (AVMs) successfully treated with

thalidomide after endoscopic, haemostatic and hormonal treatment failed. A 67-year-old man with congenital FV deficiency was seen at our institution in 2005 with recurrent GI bleeding. His lifelong history of mild bleeding had worsened over the preceding 10 years, and the selleckchem bleeding did not respond to treatment with FFP. His laboratory data are summarized in Table 1. His FV inhibitor value was initially 17 Bethesda units. This was a new finding corresponding to an acquired inhibitor. Additional testing revealed normal von Willebrand antigen. Extended oesophagogastroduodenoscopy revealed several AVMs in the duodenum that were successfully treated with argon plasma cauterization. Small non-bleeding AVMs were seen in the jejunum and were also treated with argon plasma

cauterization. He received recombinant activated factor VII and aminocaproic acid for management of haemostasis. Pulse intravenous glucocorticoids (two doses of 1 g methylprednisolone) followed by high-dose oral prednisone (1 mg kg−1) were given to treat the FV inhibitor. In the following year, the patient had three episodes of major bleeding requiring hospitalization 上海皓元医药股份有限公司 and was maintained on prophylactic and on-demand activated prothrombin complex concentrate (FEIBA), but still required 26 units of packed red blood cells (pRBCs). Corticosteroids were tapered and stopped after 4 months when his FV inhibitor titre decreased to 4 Bethesda units. In 2006, in an effort to decrease pRBC transfusion and FEIBA requirements, danazol was started at a dosage of 100 mg day−1 with progressive titration to 500 mg day−1. An 8-month period free of major bleeding without FEIBA prophylaxis was achieved with this therapy (Table 2).