Successful management of severe refractory acquired immune bleeding disorder: Prior to insisting surgery.

INTRODUCTION: Acquired bleeding disorders are rare and may be missed before surgery. Additionally, they may be refractory to conventional treatments. PRESENTATION OF CASE: A 50-year-old patient experienced prolonged post-operative bleeding when his bleeding disorder was missed prior to his undergoing inguinal herniorrhaphy. Post-operative investigations revealed severe acquired von Willebrand syndrome associated with a monoclonal gammopathy of undetermined significance. A few months later, he required umbilical herniorrhaphy, but he did not respond to attempts to raise his von Willebrand factor antigen and activity levels using conventional therapies, including desmopressin, cryoprecipitate, intravenous immunoglobulin, and Von Willebrand factor concentrate. A triple therapy combination of dexamethasone, intravenous immunoglobulin, and mycophenolate mofetil was administered, with a successful and sustained response, lasting about 2 months. The surgery was performed safely, without any complications. DISCUSSION: Conventional acquired von Willebrand syndrome treatment is usually aimed at replacing von Willebrand factor or stimulating its secretion from storage in endothelial cells. In the present case, the alternative treatment was directed against both the humoral and cell-mediated immune mechanisms. CONCLUSION: This case of acquired bleeding disorder showed that more attention must be given to a patient's coagulation profile, even if only very minor laboratory coagulation derangements are detected prior to surgery, to avoid missing such rare disorders. The described triple therapy demonstrated good effects and may be considered for inclusion in a controlled randomized study to determine its usefulness for other surgeries delayed due to severe acquired bleeding disorders. To the best of our knowledge, this triple combination treatment has not been previously used for the treatment of severe acquired bleeding disorders that are refractory to conventional therapies.

Haemostatic and fibrinolytic factors in men with a small abdominal aortic aneurysm.

BACKGROUND: : The presence of an abdominal aortic aneurysm (AAA) independently predicts cardiovascular disease (CVD) and its complications. Levels of plasma markers of fibrin turnover are raised in men with a large AAA (at least 5.5 cm) and predict CVD risk in healthy subjects. This study examined fibrin turnover in men with a small AAA. METHODS: : Seventy-five men with a small AAA (30-55 mm) were compared with 90 controls matched for age, sex and race. Haemostatic and fibrinolytic parameters were assessed. RESULTS: : Men with a small AAA had higher mean levels of fibrinogen (2.92 versus 2.59 g/l; P = 0.019), thrombin-antithrombin (TAT) complex (4.57 versus 1.89 ng/ml; P < 0.001), prothrombin F1 + 2 (1.13 versus 0.82 ng/ml; P = 0.004) and D-dimer (346.7 versus 120.2 ng/ml; P < 0.001). All markers correlated with maximum aortic diameter determined by ultrasonography. On multivariable regression the association between presence of an AAA and fibrinogen, TAT complex, prothrombin F1 + 2 and D-dimer levels remained significant after adjustment for confounding influences. CONCLUSION: : Fibrin turnover was increased in these men with a small AAA, independently of concomitant CVD, conventional risk factors and inflammatory markers. Enhanced fibrin turnover may contribute to the risk of cardiac complications in this group.