General Characteristics and Coagulopathies
Hemostatic disorders are divided into coagulopathies with bleeding (hemorrhagic syndrome) and thrombophilias, which feature an increased predisposition to thrombus formation. Pathogenetic factors are divided into vascular, plasma, and platelet components.
Coagulopathies with bleeding are subdivided into hereditary and acquired forms. Acquired forms frequently result from hepatocellular injury, anticoagulant therapy, vitamin C and K deficiencies, as well as high inhibitor activity or hyperfibrinolysis.
Angiopathies and Vasopathies
This group unites disorders caused by vascular wall lesions (predominantly affecting capillaries and pre-capillaries).
- Henoch-Schönlein purpura (hemorrhagic vasculitis).
- Hemorrhagic metropathies (dysovulatory purpura).
- Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease).
- Vitamin C deficiency (scurvy).
Differential diagnosis relies on bleeding time measurements, capillaroscopy, and functional tests (tourniquet test and pinch test).
Thrombocytopenias and Thrombocytopathies
Platelets (anucleated corpuscles, 2–5 µm in diameter) are formed in hematopoietic organs from megakaryocytes. Their pathologies are divided into quantitative changes and qualitative defects:
- Thrombocytopenias: Hereditary (caused by shortened platelet lifespan due to membrane or metabolic defects) and acquired (resulting from insufficient production, destruction, or intravascular thrombosis).
- Thrombocytopathies: Hereditary forms include Chediak-Higashi syndrome, von Willebrand disease, Bernard-Soulier syndrome, Wiskott-Aldrich syndrome, Fanconi anemia, and Glanzmann thrombasthenia. Acquired forms develop secondary to uremia, hematopoietic malignancies, liver cirrhosis, and disseminated intravascular coagulation (DIC).
Thrombophilias (Thrombotic Disease)
Conditions characterized by an increased tendency toward thrombosis.
- Hereditary Forms: Risk factors for migratory venous and arterial thrombosis at a young age. Major causes include deficiencies in natural anticoagulants (antithrombin III, protein C, protein S) and alterations in procoagulants.
- Acquired Forms: Develop when multiple links of hemostasis are simultaneously impaired. Contributing factors include elevated levels of factors I, VII, VIII, and XIII, endothelial injury, and states such as pregnancy, obesity, chronic kidney disease (CKD), or certain medications.