Coagulation disorders

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Coagulation full

Physiology of hemocoagulation[edit | edit source]

Hemostasis = a condition in which blood flow is ensured on the one hand, while on the other hand in case of bleeding it stops (x hemocoagulation = function of plasma coagulation factors)

  • 1) clot formation - clot
  • 2) activation - pledge (limited to the required section)
  • 3) breakdown of the clot (so as not to interfere with circulation)
  • parts:
  • primary hemostasis
  • plasma coagulation system
  • clotting inhibitors
  • fibrinolytic system
  • fibrinolysis inhibitors

Hemostasis disorders - coagulopathy[edit | edit source]

It is a bleeding condition caused by a decreased concentration or activity of plasma coagulation factors.

Congenital coagulopathy[edit | edit source]

Hemophilias[edit | edit source]

  • congenital defect f.VIII (hemophilia A)
  • congenital defect f.IX (hemophilia B)
  • recessively inherited X-linked disease, transmissible women
  • prenatal diagnostics already 10. − 12. week - a collection of chorionic villi, DNA examination in high-risk families, later blood collection from the fetal umbilical cord
  • clinical picture:
  • f.VIII ≤ 1%, f.IX ≤ 2% (spontaneous bleeding into joints, muscles)
  • f.VIII 5−10%, f.IX 2−6% (bleeding after injuries or operations)
  • f.VIII ≥ 10%, f.IX ≥ 6% (light form)
  • consequences: typical hemophilic arthropathy to joint amylosis, disability, extensive muscle hematomas, psoatic syndrome
  • lab. findings and treatment: aPTT, determination of f.VIII and f.IX; substitution and supportive treatment, prevention of consequences; the therapeutic regime in the hospital - preparation for surgery (treatment of bleeding into the GIT, CNS)
  • hemophilia C: defect f.X
  • parahemophilia: defect f.V

Von Willebrand's disease[edit | edit source]

  • autosomal inherited
  • vWF deficiency or malfunction (for platelet adhesion)
  • vWF is the carrier for f.VIII
  • vWF deficiency can lead to f.VIII deficiency
  • bleeding into the skin, mucous membranes, epistaxis, hypermenorrhea
  • prolonged bleeding after childbirth, injury, surgical intervention
  • at the same time with f.VIII deficiency - bleeding as in hemophilia
  • treatment: substitution, in mild cases vasopressin

Acquired coagulopathies[edit | edit source]

Disorder of resorption and utilization of vit. K[edit | edit source]

  • insufficient synthesis of factors
  • liver cell involvement
  • vit.K transport disorder in obstructive jaundice
  • intestinal resorption disorder
  • overdose of dicoumarin treatment (kamavitiv, frozen plasma, prothrombin complex factor concentrate)

Disseminated intravascular coagulation (DIC)[edit | edit source]

- a necessary evoking moment

  • DIC development
  • a) the coagulation system is activated (if it is not meant to be) → microthrombus formation → storage in organs
  • b) consumption of coagulation material → formation of hypocoagulation state
  • c) defibrination
  • d) fatal bleeding
  • cause: endotoxin leaching, tissue thromboplastin release, proteolytic enzyme leaching, antigen-antibody reaction
  • accompanies: obstetric complications, injuries, infections, sepsis, anaphylactic shock
  • course: activation phase (microthrombolization), compensated and decompensated phase, excessive activation phase of secondary fibrinolysis
  • treatment: substitution of all components (MP, thrombo, fibrinogen, EM), eliminate the inducing moment

Links[edit | edit source]

Related articles[edit | edit source]

Literature[edit | edit source]

PECKA, Miroslav. Laboratorní hematologie v přehledu. [Díl 2.], Fyziologie a patofyziologie krevní buňky. 1. vydání. Český Těšín : Finidr, 2006. ISBN 80-86682-00-5.