American journal of hematology
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Limited data are available regarding optimal treatment with desmopressin (DDAVP) or intermediate-purity FVIII concentrates rich in VWF (CFCs) in patients with von Willebrand disease (VWD) who undergo planned surgery. We undertook a retrospective review over 10 years (1988-1997) and identified 27 patients treated with DDAVP for 35 surgical events and 38 patients who received CFCs for 68 elective surgical events. Tranexamic acid was usually added for mucosal surgery. ⋯ For major surgery the median pre- and post-operative doses of CFCs were 54 and 43 IU/kg, respectively, and for minor surgery the median doses varied between 34 and 52 IU/kg preoperatively and between 23 and 37 IU/kg postoperatively. The effectiveness of haemostasis was excellent in 32 events (91%) treated with DDAVP and in 56 events (82%) treated with CFCs. It is concluded that patients with VWD do not carry an increased operative risk if appropriate therapy is given.
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Antiphospholipid antibodies (APA) are a common cause of acquired thrombophilia. APA recognize plasma phospholipid-binding proteins (e. g., beta(2)-glycoprotein I, prothrombin, annexin V, etc.). Catastrophic antiphospholipid syndrome (CAPS) is an uncommon variant of the antiphospholipid syndrome. ⋯ Increasingly, infections are recognized as a major precipitating condition. The majority of patients present with renal involvement as well as evidence of acute respiratory distress syndrome (ARDS). This review discusses the clinical and pathophysiologic aspects of CAPS as well as the differenital diagnosis.
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Disseminated intravascular coagulation (DIC) is a complex acquired coagulopathy resulting from excessive thrombin formation. Abnormal tissue factor (TF) expression is a major mechanism initiating DIC in many disorders, including obstetrical complications, sepsis, cancer, and trauma. ⋯ Treatment of DIC should focus on reversing the underlying disorder initiating the coagulopathy. Novel treatments are being investigated for treating DIC; many of these experimental modalities target the excessive TF activity that characterizes DIC.
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Although excessive bleeding is widely recognized as a common complication of cardiac surgery, the recent success of antifibrinolytic drugs as prophylactic hemostatic agents has received little attention outside the surgical literature. The etiology of the coagulopathy following cardiac surgery is clearly multifactorial; however, the success of antifibrinolytic drugs as hemostatic agents suggests that fibrinolysis contributes to bleeding in this setting. Increasingly widespread administration of these drugs necessitates increased awareness of the risks and benefits posed by perioperative antifibrinolytic therapy. The objectives of this review are to understand the mechanisms of action of antifibrinolytic drugs in the context of the normal hemostatic response and to review evidence pertaining to the efficacy and safety of antifibrinolytic drugs as hemostatic agents during cardiac surgery.
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In summary, LAP is an intriguing enzyme and its control is related to pituitary-adrenal function. A review of the changes in LAP activity which occur in some physiological conditions and in disease states has been presented. The function of LAP, however, is unknown. ⋯ Of prime importance is the differentiation of CML from a leukemoid reaction or agnogenic myeloid metaplasia with a leukocytosis. However, in no instance is the LAP value alone diagnostic of any disease. It remains a laboratory test to be utilized in conjunction with all other available clinical data.