Coagulation disorders

Coagulation full

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.

Congenital coagulopathy

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 - 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

• 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

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

Links

Related articles

• Blood
• Vitamin 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.