Seminars in hematology
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Seminars in hematology · Jan 2004
ReviewChallenges for providing effective hemostasis in surgery and trauma.
Vascular injury, whether surgical or traumatic, triggers a complex series of regulatory events. The understanding of these events, their interdependence, and their effect on hemostasis and thrombosis, is slowly being unraveled. The current understanding of these processes is reviewed in this paper. ⋯ Hence, the initial treatment of patients with severe hemorrhage relies on improving the patient's physiological status and on basic surgical techniques. Should these efforts fail, then a number of topical hemostatic agents, selective inhibitors of fibrinolysis, and procoagulant molecules, such as recombinant factor VIIa, may be utilized. However, many of these agents have not yet been tested in clinical trials and studies are urgently needed to determine efficacy, safety, optimal dosage and time of administration.
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Seminars in hematology · Jan 2004
Reviewvon Willebrand factor, ADAMTS-13, and thrombotic thrombocytopenic purpura.
Thrombotic thrombocytopenic purpura (TTP) is a severe, occlusive, microvascular "thrombotic microangiopathy" characterized by systemic platelet aggregation, organ ischemia, profound thrombocytopenia, and erythrocyte fragmentation. Failure to degrade "unusually large" (UL) von Willebrand factor (VWF) multimers as they are secreted from endothelial cells probably causes most cases of familial TTP, acquired idiopathic TTP, thienopyridine-related TTP, and pregnancy-associated TTP. The emphasis in this review is the pathophysiology of familial and acquired idiopathic TTP. ⋯ In familial TTP, mutations in the ADAMTS13 gene cause absent or severely reduced plasma VWF-cleaving metalloprotease activity. Acquired idiopathic TTP, in contrast, is the result in many patients of the production of autoantibodies that inhibit the function of ADAMTS-13. Established, evolving, and some of the unresolved issues in TTP pathophysiology will be summarized.
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Seminars in hematology · Jan 2004
ReviewStrategies for minimizing blood loss in orthopedic surgery.
Several major orthopedic surgical procedures including hip arthroplasty, femoral osteotomy, and spinal fusion may result in significant blood loss and the need for allogeneic blood transfusions. Due to the heightened awareness of the potential deleterious effects of allogeneic blood product administration, several techniques have been evaluated to determine their efficacy in limiting perioperative blood loss. ⋯ These techniques include: general considerations, autologous transfusion therapy, intraoperative and postoperative blood salvage, pharmacologic manipulation of the coagulation cascade, and controlled hypotension. Undoubtedly, many of these techniques are effective alone; however, the goal of performing major orthopedic surgical procedures without the use of allogeneic blood products can only be accomplished by combining several of these techniques.
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Seminars in hematology · Jan 2004
ReviewThe management of coagulopathy and blood loss in liver surgery.
Liver surgery has long been associated with massive perioperative blood loss and high rates of postsurgery morbidity and mortality. Recent advances in our knowledge of hepatic segmental anatomy have led to the evolution of liver resection, and a growing awareness of the coagulopathy present in cirrhotic patients has produced a greater understanding of the factors influencing surgical hemostasis. ⋯ The potential role of recombinant factor VIIa (rFVIIa) in providing safe hemostasis during such procedures will also be explored. Today, due to careful monitoring and correction of coagulopathy, improved surgical techniques, and judicious patient selection, liver surgery is no longer a high-risk specialty with an unfavorable risk profile, but a safe and widely practiced procedure.
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Seminars in hematology · Jan 2004
von Willebrand factor-cleaving protease (ADAMTS-13) activity determination in the diagnosis of thrombotic microangiopathies: the Swiss experience.
Severe deficiency of von Willebrand factor (VWF)-cleaving protease (ADAMTS-13) activity (<5% of normal) is a specific finding for acute idiopathic thrombotic thrombocytopenic purpura (TTP), a disorder that presents as thrombocytopenia, microangiopathic hemolytic anemia, and often organ dysfunction such as neurological disturbances or renal failure, and fever. Between January 2001 and July 2003, ADAMTS-13 activity was determined in plasma samples of 396 consecutive patients referred to our laboratory for diagnostic purposes. Plasma samples with ADAMTS-13 activity less than 5% were in addition tested for the presence of inhibitory antibodies. ⋯ Forty-three (62%) patients with ADAMTS-13 activity less than 5% displayed inhibitory antibodies. Severe ADAMTS-13 deficiency was found in 60% of patients diagnosed with acute idiopathic TTP, but in none of 130 patients diagnosed with HUS or in any of the 14 patients with hematopoietic stem cell transplantation-associated TMA. Thus, plasma ADAMTS-13 activity less than 5% does not identify all patients clinically diagnosed with TTP, and severe ADAMTS-13 deficiency is not invariably associated with clinical manifestations of microvascular platelet clumping.