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Coagulation Disorders in Patients with Cirrhosis

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Complications of Cirrhosis

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Abstract

Coagulation abnormalities have been a hallmark of the protein synthetic dysfunction of cirrhosis since early physicians noted increased bleeding tendencies in the jaundiced patient. During the twentieth century, an increasing fund of knowledge regarding inborn coagulation defects led to elucidation of the coagulation protein cascade and basic laboratory tests describing prolonged clotting times in diseases such as the hemophilias. Prolonged prothrombin times and bleeding times in cirrhosis patients led physicians and scientists to the conclusion that these patients were at greatly increased risk for bleeding and were therefore “auto-anticoagulated.” Despite these laboratory findings, the usual cirrhosis patient does not suffer from spontaneous bleeding related to coagulation abnormalities like hemophilia patients and in fact many cirrhosis patients are prone to thrombosis events and thrombophilia. Investigation into this paradox in recent years has led to new discoveries that stable cirrhosis patients are in a “rebalanced” state of hemostasis characterized by equal and opposite compensatory mechanisms in primary hemostasis, coagulation, and fibrinolysis. A major difficulty in the clinical care of cirrhosis patients is the lack of adequate and accessible clinical laboratory assays to give an overview of the hemostasis pattern of an individual patient. Consequently, clinical decisions based solely on tests designed for congenital clotting disorders are wrought with dangers. It is now understood that despite this rebalance, the reserve is tenuous and perturbations due to factors such as infection, kidney disease, surgical procedures, mechanical sources of bleeding, and medications can rapidly tilt the balance away from hemostasis. This chapter briefly describes the pathophysiology of the rebalanced hemostasis system in cirrhosis patients, the current clinically available laboratory assays to describe the hemostatic disorders, and the established and emerging therapies for bleeding and clotting disorders in patients with cirrhosis.

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Abbreviations

aPC:

Activated protein C

FFP:

Fresh frozen plasma

INR:

International normalized ratio

LMWH:

Low molecular weight heparin

MELD:

Model for end-stage liver disease

PCC:

Prothrombin complex concentrates

PT:

Prothrombin time

PVT:

Portal vein thrombosis

rFVIIa:

Recombinant activated factor VII

ROTEM:

Rotational thromboelastometry

TEG:

Thromboelastography

VTE:

Venous thromboembolism

vWF:

Von Willebrand factor

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Authors and Affiliations

  1. Division of Gastroenterology and Hepatology, University of Virginia, JPA and Lee Street, MSB 2145, PO Box 800708, Charlottesville, VA, USA

    Patrick G. Northup MD, MHS, Nicolas M. Intagliata MD & Neeral L. Shah MD

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  1. Patrick G. Northup MD, MHS
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  2. Nicolas M. Intagliata MD
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  3. Neeral L. Shah MD
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Correspondence to Patrick G. Northup MD, MHS .

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Editors and Affiliations

  1. Department of Transplant, Mayo Clinic, Jacksonville, Florida, USA

    Andrew P. Keaveny

  2. Institut de Malalties Digestives i Metaboliques, University of Barcelona, Barcelona, Spain

    Andrés Cárdenas

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Northup, P., Intagliata, N., Shah, N. (2015). Coagulation Disorders in Patients with Cirrhosis. In: Keaveny, A., Cárdenas, A. (eds) Complications of Cirrhosis. Springer, Cham. https://doi.org/10.1007/978-3-319-13614-1_21

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