Within the wider context of the physiology of blood, the science of hemostaseology focuses specifically on blood clotting events, inhibition of the coagulation pathway, and dissolution of existing blood clots. Research topics in this area include:

1. The complete clotting process with all the participating coagulating factors, which mutually regulate each other to trigger a coagulation cascade
2. Synthesis of coagulation factors in the vascular endothelium
3. The role of platelets (thrombocytes) in hemostasis (clotting)

Hemostaseology deals with the complex interactions between blood vessels, coagulation factors, and blood cells. Rudolf Marx, who co-founded this discipline, defined hemostaseology as the ‘slowing down and stopping’ of the blood.

The process of coagulation is divided into the following three phases: vasoconstriction, primary hemostasis, and secondary hemostasis. A detailed description of the mechanism of the coagulation cascade can be found in the article: The biochemistry of blood.

Bleeding and blood coagulation disorders are more commonly known as ‘hemophilia’. They alter the bleeding type pathologically, i.e., an extended period of time until coagulation occurs, which depends on the severity of the case and is managed therapeutically via medications. Blood coagulation disorders can be hereditary or result from other causes.

Hemophilia Types A and B:

Hemophilia is an X-linked recessive genetic disorder, which explains why more males than females are affected: The defect in female patients is canceled by the presence of a second X chromosome in female genes.

The condition can be distinguished by the lack of a protein, a coagulation factor, which under specific physiological conditions, promotes blood coagulation and thus facilitates wound healing following an injury.

Epidemiology of Hemophilia Types A and B:

Hemophilia A, where coagulation factor VIII is missing, accounts for 80% of cases. In the remaining 20% of hemophilia B cases, the coagulation factor IX is the missing factor. The signal cascade of intrinsic coagulation is, therefore, not fully functional, suggesting that the formation of a fibrin framework and subsequent clotting is not possible. This phenomenon significantly increases bleeding times and impairs wound healing.

Treatment of Hemophilia A and B:

Substitution Therapy for Hemophilia A and B-

As a prophylactic treatment, patients are injected intravenously with recombinant coagulation factor VIII.

The manufacture of the protein comprising 2,200 amino acids, is a synthetic challenge for clinical chemists, due to the short shelf life of the protein in the serum. Currently, the coagulation factor is synthesized as a powder and is only combined with the serum and injected as needed.

Media Contact: 
Allison Grey 
Journal Manager 
Journal of Clinical chemistry and Laboratory Medicine
Email: jcclm@molecularbiol.com