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The estimated incidence of congenital factor VII deficiency is 1:500 000. Severe FVII deficiency is associated with spontaneous bleeding such as intraarticular or intracranial haemorrhage. The risk of perioperative bleeding is high during cardiac surgery as a result of the exposure to extracorporeal circulation, systemic anticoagulation, loss of coagulation factors, and postoperative platelet malfunction. Effective treatment of pre-existing coagulopathy is crucial, as increased morbidity and mortality are associated with allogenic blood transfusions. We report a 67-year-old Caucasian male patient with severe congenital FVII deficiency, undergoing successful and uneventful elective mitral valve repair surgery, radiofrequency epicardial atrial fibrillation ablation, and exclusion of the left atrial appendage. He presented with severe symptomatic mitral valve regurgitation, moderate pulmonary artery hypertension, and paroxysmal atrial fibrillation; his left ventricular ejection fraction was 67%. Three years before surgery, during a routine assessment of a grade I renal failure, a spontaneous International Normalised Ratio of 4.1 was observed. He had no history of previous spontaneous bleeding. The diagnosis of a severe FVII deficiency, with an FVII activity below 2% (normal references values in City Hospital Triemli Zurich: 55-170%) was made. [ABSTRACT FROM AUTHOR]

Principles: High-dose chemotherapy with subsequent autologous stem cell transplantation (ASCT) is an important treatment option in younger patients with multiple myeloma (MM). We analysed the outcome of patients treated at our institution outside the clinical trials framework and tried to identify risk factors prognostic for survival., Methods: Medical histories of the patients were screened for response, event-free survival (EFS) and overall survival (OS). Pre-transplant variables were analysed to identify possible prognostic risk factors., Results: Overall, 182 ASCT were performed in 120 patients with MM from 2002 to 2007. Treatment-related mortality (TRM) was 0.5%. Median EFS was 23.1 months (95% confidence interval [CI]: 19.4-28.4) and median OS was 49.8 months (95%CI: 43.7 - not reached) in the whole patient population. The median OS in patients who received one ASCT was 46.4 months (95%CI: 35.2 - not reached), and 63.7 months (95%CI: 48.9 - not reached) in patients who underwent double ASCT. Patients who already achieved a complete remission (CR) before ASCT had a longer EFS (p = 0.016) than patients without CR. Additionally, patients who achieved a CR after ASCT had a longer EFS (p = 0.0061) and OS (p = 0.0024) than patients without CR. ISS stage