coagulation disorder; coagulopathy
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Etiology
- hemophilia
- vitamin K deficiency
- coagulation factor inhibitor - mimics hemophilia
- disseminated intravascular coagulation
- localized intravascular coagulation
- liver dysfunction
- associated with pathologic thrombosis, fibrinolysis or both
- prolongs PT, then aPTT
- eventually dysfibrinogenemia occurs
- factor V in plasma is low, factor VII in plasma is low
- factor VIII in plasma is high (synthesized by vascular endothelium & cleared by liver)
- D-dimer in plasma may be elevated (cleared by liver)
- plasma fibrinogen is low
Clinical manifestations
(coagulation defect)
- delayed bleeding after trauma
- bleeding into deep joints, muscle, retroperitoneum
- hematomas & hemarthrosis
Laboratory
- prothrombin time (PT) & activated partial thromboplastin time (aPTT)
- mixing study if prolonged aPTT to distinguish coagulation factor deficiency from coagulation factor inhibitor (deficiency completely corrects)
- thrombin time: conversion of fibrinogen to fibrin
- D-dimer in plasma identifies disseminated intravascular coagulation associated with excessive fibrinolysis[1]
- factor VIII activity in plasma & factor VIII antigen in plasma not useful clinically in establishing coagulopathy of liver disease from disseminated intravascular coagulation[2]
Differential diagnosis
- prolonged PT, normal aPTT
- normal PT, prolonged aPTT
- prolonged PT & aPTT
- deficiency of specific coagulation factors
- coagulation factor II
- coagulation factor V
- coagulation factor X
- fibrinogen deficiency
- severe liver disease
- disseminated intravascular coagulation
- vitamin K deficiency
- heparin overdose
- deficiency of specific coagulation factors
- normal PT & aPTT
Management
- use fresh frozen plasma to replace coagulation factors
- fresh frozen plasma not needed if INR < 1.9[2]
- use cyroprecipitate* as an adjunct to fresh frozen plasma
* cyroprecipitate contains factor VIII, vWF, fibrinogen used mainly for fibrinogen replacement in disseminated intravascular coagulation[2]
More general terms
More specific terms
- coagulation factor antibody
- coagulation factor deficiency
- coagulopathy of liver disease
- hypercoagulability
- hypofibrinogenemia
- localized intravascular coagulation
- thrombotic disorder
Additional terms
- coagulation
- platelet disorder; thromboasthenia
- risk for hemorrhage; hemorrhagic diathesis; bleeding disorder
References
- ↑ 1.0 1.1 Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 321
- ↑ 2.0 2.1 2.2 2.3 Medical Knowledge Self Assessment Program (MKSAP) 14, 17, 19 American College of Physicians, Philadelphia 2006, 2015, 2022
Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022 - ↑ Tripodi A, Primignani M, Chantarangkul V et al An imbalance of pro- vs anti-coagulation factors in plasma from patients with cirrhosis. Gastroenterology. 2009 Dec;137(6):2105-11 PMID: https://www.ncbi.nlm.nih.gov/pubmed/19706293
- ↑ ARUP Consult: Uncommon Factor Deficiencies The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/factor-deficiencies-uncommon