Coagulation disorders

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

Hemophilias

 * 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 - 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; 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

 * 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

Disorder of resorption and utilization of vit. K

 * 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)
- 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

Related articles

 * Blood
 * Vitamine K
 * Hemostasis disorders
 * Hemocoagulation

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