Management of disseminated intravascular coagulation associated with aortic aneurysm and vascular malformations
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PROGRESS IN HEMATOLOGY Advances in diagnosis and treatment of disseminated intravascular coagulation
Management of disseminated intravascular coagulation associated with aortic aneurysm and vascular malformations Shinya Yamada1 · Hidesaku Asakura1 Received: 29 September 2020 / Revised: 4 October 2020 / Accepted: 22 October 2020 © Japanese Society of Hematology 2020
Abstract Aortic aneurysms and vascular malformations are sometimes associated with disseminated intravascular coagulation (DIC). A typical blood coagulation test shows decrease in platelet count and fibrinogen, and increases in fibrin/fibrinogen degradation products (FDP) and D-dimer. The coagulation activation marker thrombin–antithrombin complex (TAT) and the fibrinolysis activation marker plasmin-α2 plasmin inhibitor (PIC) are significantly increased. α2 plasmin inhibitor (α2PI) is significantly reduced. Since no prolongation of prothrombin time (PT) is noticeable and activated partial thromboplastin time (APTT) is shortened in some cases, DIC cannot be diagnosed or ruled out by PT and APTT alone. The cornerstone of treatment for DIC is to treat the underlying disease. However, surgery is not possible in some cases. Follow-up may be appropriate in patients with abnormal results from coagulation tests and no bleeding. However, pharmacotherapy is often required in cases with bleeding. Unfractionated heparin, low molecular weight heparin, protease inhibitors, recombinant thrombomodulin, direct oral anticoagulants, and factor XIII preparations are effective. If PIC is significantly increased and α2PI is significantly decreased, or if the bleeding is severe, tranexamic acid is used as an antifibrinolytic therapy with anticoagulant therapy. In such cases, attention should be paid not only to TAT but also changes in PIC. Keywords Aortic aneurysm · Vascular malformations · Disseminated intravascular coagulation · Fibrinolytic activation · Direct oral anticoagulant (DOAC)
Introduction The normal arterial wall comprises a three-layered structure from the intravascular lumen to the intima, media, and adventitia. The aorta is an elastic-type artery in which the intima comprises vascular endothelial cells, a subendothelial layer, and an internal elastic lamina composed of elastic fibers. The media is the thickest layer of the aortic wall, comprising 40–70 layers of fenestrated elastic membrane, vascular smooth muscle, and external elastic lamina. The adventitia is composed of fibroblasts, collagen fibers, and vasa vasorum, and is continuous with the surrounding connective tissue.
* Shinya Yamada [email protected] 1
An aneurysm in which an artery expands while maintaining the three-layered structure of intima, media, and adventitia is called a true aneurysm. When formed by only some of the three layers (e.g., only the adventitia or only the adventitia and media), the pathology is known as a pseudoaneurysm. Aneurysms are also classified as spindle-shaped or saccular according to the overall morphology (Fig. 1). Thoracic aortic aneurysms are classifi
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