Gross hematuria due to acquired haemophilia in hereditary hemorrhagic telangiectasia.

Hereditary haemorrhagic telangiectasia (HHT, Osler-Weber-Rendu syndrome) is a rare inherited disease; acquired haemophilia, caused by factor VIII inhibitors, is a rare autoimmune disorder. Both diseases cause bleeding manifestations. We report a case of a young woman affected by hereditary haemorrhagic telangiectasia who was admitted to our unit because of gross hematuria. The prolonged activated partial thromboplastin time suggested us the possibility of a bleeding cause other than hereditary haemorrhagic telangiectasia. The test result for factor VIII inhibitors resulted positive and a diagnosis of acquired haemophilia was made. The patient was treated with frozen fresh plasma and with activated eptacog alpha and a steroid therapy was started. After she received steroid, factor VIII inhibitor titre decreased and activated partial thromboplastin time gradually recovered. After 2 weeks a complete normalization of activated partial thromboplastin time was obtained. Acquired haemophilia is a life-threatening disorder. Measuring factor VIII activity and its inhibitor in patients with bleeding and a prolonged activated partial thromboplastin time are recommended even in patients affected by other known hemorrhagic disease.

Juxta-anastomotic stenosis of native arteriovenous fistulas: surgical treatment versus percutaneous transluminal angioplasty.

PURPOSE: Juxta-anastomotic stenosis (JAS) is a complication of arteriovenous fistulas (AVFs). Both surgical revision (SR) and percutaneous transluminal angioplasty (PTA) may correct JAS. In this study we compare the results for SR treatment versus PTA. METHODS: From January 2005 until December 2008, 66 PTA and 68 SR were performed in 43 and 57 uremic pts with JAS of the native AVF, respectively. Efficacy of SR and PTA was evaluated measuring brachial arterial flow (BAF) by CDU. The Kaplan-Meier table of primary and assisted primary patency was analyzed. RESULTS: PTA was attempted in 50 patients. PTA failed in 7 patients and they were switched to SR. In 43 pts, PTA produced a favorable effect, with a mean increase of 99 ± 70% (p<0.001) in blood flow. Restenosis occurred in 17 pts: 2 were treated by SR and 15 by PTA. Restenosis occurred again in 6/15 pts: after second restenosis, 5/6 pts received a third PTA with stenting, 1 patient underwent SR. The failure of access occurred after 12-17 months in 3 pts. In 57/57 JAS treated by SR, a new well-functioning fistula was created upstream of the stenosis, with a mean increase of 102 ± 71% in blood flow (p<0.001). Restenosis occurred in 15 pts: 9 were treated by SR and 6 by PTA. Access failure occurred after 3-36 months in 9 pts. The Kaplan-Meier table showed a better primary patency for SR (p<0.05) without difference in assisted primary patency. CONCLUSIONS: SR showed a better primary patency then PTA, confirming the trend to stenosis relapse after PTA. So, as PTA does not exclude a later correction of the JAS, the similar assisted patency suggests to perform a PTA first, reserving SR for after its failure.