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coagulation disorder; coagulopathy
Etiology:
1) hemophilia
2) vitamin K deficiency
3) coagulation factor inhibitor - mimics hemophilia
4) disseminated intravascular coagulation
5) localized intravascular coagulation
6) liver dysfunction
a) associated with pathologic thrombosis, fibrinolysis or both
b) prolongs PT, then aPTT
c) eventually dysfibrinogenemia occurs
d) factor V in plasma is low, factor VII in plasma is low
e) factor VIII in plasma is high (synthesized by vascular endothelium & cleared by liver)
f) D-dimer in plasma may be elevated (cleared by liver)
g) plasma fibrinogen is low
Clinical manifestations: (coagulation defect)
1) delayed bleeding after trauma
2) bleeding into deep joints, muscle, retroperitoneum
3) 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
- coagulation factor deficiency or coagulation factor inhibitor
- disseminated intravascular coagulation
- liver disease
- vitamin K deficiency
- warfarin
- normal PT, prolonged aPTT
- deficiency of specific coagulation factors
- coagulation factor VIII deficiency
- coagulation factor IX deficiency
- coagulation factor XI deficiency
- coagulation factor XII deficiency
- von Willebrand disease
- heparin
- 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
- warfarin
- heparin overdose
- normal PT & aPTT
- platelet disorder
- von Willebrand disease (coagulation factor VIII adequate)
- scurvy
- Ehlers-Danlos syndrome
- hereditary hemorrhagic telangiectasia
- coagulation factor XIII deficiency
Management:
1) use fresh frozen plasma to replace coagulation factors
- fresh frozen plasma not needed if INR < 1.9 [2]
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]
Related
coagulation
platelet disorder; thromboasthenia
risk for hemorrhage; hemorrhagic diathesis; bleeding disorder
Specific
coagulation factor antibody
coagulation factor deficiency
coagulopathy of liver disease
hypercoagulability
hypofibrinogenemia
localized intravascular coagulation
thrombotic disorder
General
circulatory system disease
hematologic disease (blood disorder, blood dyscrasia)
References
- Harrison's Principles of Internal Medicine, 13th ed.
Isselbacher et al (eds), McGraw-Hill Inc. NY,
1994, pg 321
- 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: 19706293
- ARUP Consult: Uncommon Factor Deficiencies
The Physician's Guide to Laboratory Test Selection & Interpretation
https://www.arupconsult.com/content/factor-deficiencies-uncommon