PO66 | Viscoelastic tests (ROTEM) in disseminated intravascular coagulation associated with aortic aneurysm

Am. Pizzini¹, C. Biasoli², M. Bovara³, E. Gesuete¹, A. Fusco¹, C. Fantoni¹, P. Pedrazzi², R. Mancini³, M. Silingardi¹ | ¹Ambulatorio Emostasi & Trombosi, Dipartimento Medico, Ospedale Maggiore AUSL Bologna; ²SSI Presa in Carico delle Malattie Emorragiche e Tromboemboliche della Romagna; ³Laboratorio Unico Metropolitano AUSL Bologna, Italy

Background: Aortic aneurysms can be complicated by disseminated intravascular coagulation (DIC) with a predominantly fibrinolytic type in which both coagulation and fibrinolysis are simultaneously activated. The procoagulant state may contribute to microvascular and macrovascular thrombosis but in “enhanced-fibrinolytic-type DIC” multiple fibrin clots dissolve one after other due the hyperfibrinolysis. As a result, ischemic organ damage due to multiple microthrombosis is rarely seen as a clinical manifestation; in contrast, severe bleeding symptoms are more likely to occur with the dissolution of the thrombi. Characteristic laboratory findings include a low platelet (PTL) count, a normal-to-prolonged prothrombin time (PT) and a shortened-to-prolonged activated partial thromboplastin time (aPTT). Due to the enhanced fibrinolysis, the D-Dimer is increased (are especially high the Fibrinogen degradation products, FDP) and Fibrinogen levels are consequently decreased. In addition levels of Plasminogen Activator Inhibitor-1 (PAI-1 a fibrinolytic inhibitor) are normal or only mildly elevated. Concentrations of α2plasmin-inhibitor (α2PI) are markedly decreased and when they are less than 50%, caution should be taken against major bleeding. Fibrinogen levels are also markedly decreased in typical cases, not only because of the consumption associated with the dissolution of multiple microthrombi, but also because of the degradation of Fibrinogen by Plasmin.

Cases Report: We describe two cases of “enhanced-fibrinolytic-type-DIC” associated with aortic thrombosed aneurysms. Both patients presented with bleeding symptoms (spontaneous large limbs hematomas and anemia) associated with marked reduction in Fibrinogen (<100 mg/dl), prolonged PT/aPTT, mild thrombocytopenia and elevated D-dimer. The “enhanced-fibrinolytic-type-DIC” diagnosis was made even through viscoelastic tests (ROTEM). The Table 1 shows the clinical and laboratory findings of the patients. The cornerstone of DIC treatment is the treatment of the underlying pathology. However in both patients the surgery was not feasible. Therefore the therapeutic approach was to restore Fibrinogen and antifibrinolytic therapy in the acute phase of haemorrhage and antiplatelets/anticoagulant therapy once the bleeding is resolved.

Conclusions: It is known that some vascular diseases such as hemangiomas and vascular malformation (Kasabach-Merritt syndrome, Klippel-Trenaunay-Weber syndrome) can be associated with a chronic hyperfibrinolytic DIC and also in some aortic aneurysms, especially if large or complicated (with endoleak or thrombosis). In “enhanced-fibrinolytic-type DIC” associated with aortic aneurysms activation of coagulation factors and plasmin are responsible for both the state of hypercoagulability and fibrinolysis, with increased thrombotic or bleeding risk. This process can be dynamic and may require simultaneous haemostatic-replacement, but also anticoagulant or antiplatelet treatment in combination or later. The use of the ROTEM in both cases confirmed the hyperfibrinolysis state and allowed the correct administration of Fibrinogen and Antifibrinolytic therapy, proving to be a valid aid even outside the his codified use sets (polytrauma, post-partum hemorrage, cardiac surgery and liver transplant).