Diseases of the blood (hematology)

Hemophilia: causes, symptoms, diagnosis, treatment

Hemophilia is usually a congenital disorder caused by a deficiency of factors VIII or IX. The severity of the factor deficiency determines the likelihood and severity of bleeding. Bleeding into soft tissue or joints usually occurs within hours of injury.

Disseminated intravascular coagulation syndrome (DIC): causes, symptoms, diagnosis, treatment

Disseminated intravascular coagulation syndrome (DIC, consumption coagulopathy, defibrination syndrome) is a disorder with pronounced generation of thrombin and fibrin in circulating blood.

Coagulation disorders due to circulating anticoagulants: causes, symptoms, diagnosis, treatment

Circulating anticoagulants are usually autoantibodies that neutralize specific coagulation factors in vivo (e.g., autoantibodies against factors VIII and V) or inhibit protein-bound phospholipids in vitro. Sometimes late-type autoantibodies cause bleeding in vivo by binding prothrombin.

Blood clotting disorder

Pathological bleeding may occur as a result of diseases of the blood coagulation system, platelets or blood vessels. Coagulation disorders may be acquired or congenital.

Hyperhomocysteinemia: causes, symptoms, diagnosis, treatment

Hyperhomocysteinemia may contribute to the development of arterial or venous thromboembolism, possibly due to damage to the endothelial cells of the vessel wall. Plasma homocysteine levels are increased more than 10-fold in homozygotes with cystathionine synthase deficiency.

Antithrombin deficiency: causes, symptoms, diagnosis, treatment

Antithrombin is a protein that inhibits thrombin and factors Xa, IXa, Xla. The prevalence of heterozygous plasma antithrombin deficiency is 0.2 to 0.4%. Half of heterozygous individuals develop venous thrombosis.

Protein Z deficiency: causes, symptoms, diagnosis, treatment

Protein Z is a vitamin K-dependent protein that functions as a cofactor in the process of inhibiting blood coagulation by forming a complex with plasma protein Z-dependent protease inhibitor.

Protein C deficiency: causes, symptoms, diagnosis, treatment

Since activated protein C causes the breakdown of factors Va and VIIIa, it is thus a natural plasma anticoagulant. A decrease in protein C due to genetic or acquired causes provokes the occurrence of venous thrombosis.

Factor V resistance to activated protein C: causes, symptoms, diagnosis, treatment

Activated protein C cleaves factors Va and VIIIa, thereby inhibiting the blood clotting process. Any of several mutations of factor V causes its resistance to activated protein C, thereby increasing the susceptibility to thrombosis. The most common mutation of factor V is the Leiden mutation. Homozygous mutations increase the risk of thrombosis to a greater extent than heterozygous mutations.

Thrombophilia: causes, symptoms, diagnosis, treatment

In healthy individuals, hemostatic balance is the result of the interaction of procoagulant (promoting clot formation), anticoagulant and fibrinolytic components.

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