Hemorrhagic diatheses

General characteristics and coagulopathies

Hemostatic disorders are divided into coagulopathies with bleeding (hemorrhagic syndrome) and thrombophilias, which are characterized by an increased tendency to thrombosis. Pathogenetic factors are divided into vascular, plasma, and platelet factors.

Coagulopathies with bleeding are divided into hereditary and acquired. Acquired forms often arise due to hepatic parenchyma lesions, anticoagulant therapy, vitamin C and K deficiencies, as well as high inhibitor activity or hyperfibrinolysis.

Angiopathies and vasopathies

This group unites diseases caused by damage to the vascular wall (primarily of the capillary and precapillary type).

• Hemorrhagic vasculitis.
• Hemorrhagic metropathies (dysovarian purpura).
• Hemorrhagic angiomatosis (Rendu — Osler disease, telangiectasias).
• Vitamin C deficiency.

For differential diagnosis, determination of bleeding time, capillaroscopy, and functional tests (tourniquet test and pinch test) are used.

Thrombocytopenias and Thrombocytopathies

Platelets (anucleate corpuscles, 2–5 µm in diameter) are formed in hematopoietic organs from megakaryocytes. Their pathologies are divided into quantitative changes and qualitative defects:

1. Thrombocytopenias: hereditary (caused by shortened platelet lifespan due to membrane or energetic defects) and acquired (due to insufficient production, destruction, or intravascular thrombosis).
2. Thrombocytopathies: hereditary forms include Chediak — Higashi syndrome, von Willebrand disease, Bernard — Soulier thrombodystrophy, Wiskott — Aldrich syndrome, Fanconi syndrome, and Glanzmann thrombasthenia. Acquired forms develop against the background of uremia, hematopoietic tissue tumors, liver cirrhosis, and DIC syndrome.

Thrombophilias (Thrombotic disease)

Conditions with an increased tendency to thrombosis.

• Hereditary forms: risk factor for migrating venous and arterial thrombosis at a young age. The main causes are associated with anticoagulant deficiency (antithrombin III, protein C, protein S) and alterations in procoagulants.
• Acquired forms: occur with simultaneous damage to various links of hemostasis. Contributing factors include an increase in factors I, VII, VIII, XIII, vascular wall damage, as well as conditions such as pregnancy, obesity, chronic kidney disease, or the intake of certain medications.

Class and exam questions

What else to study on this topic

• Iatrogenic hemorrhagic syndromes
• Wiskott — Aldrich syndrome and other hereditary thrombocytopathies
• The role of antithrombin III, proteins C and S in thrombophilias
• Functional tests for assessing vascular state (tourniquet and pinch tests)
• Pathogenetic differences between DIC syndrome and isolated coagulopathies

Other topics in Hematopoietic System Diseases

Prepared by WhiteCoat Pro faculty in accordance with the medical school curriculum. For educational purposes only.