Epistaxis

Epistaxis in Werlhof's disease

Idiopathic autoimmune thrombocytopenic purpura (Werlhof's disease) is a congenital pathology characterized by alternating periods of remission and exacerbation. It is based on the production of specific autoantibodies that purposefully destroy the patient's own platelets, leading to a severe disturbance of hemocoagulation.

• Anamnesis: A tendency toward persistent epistaxis is observed in patients from early childhood. Concurrently, there is increased bleeding from minor cuts, bruises, and after tooth extractions. A key diagnostic sign in girls is that menarche immediately transitions into massive bleeding.
• Objective picture: Examination reveals multiple petechiae and bruises on the skin.
• Laboratory markers: The platelet count critically drops below 70–100 × 10⁹/L. Blood clotting time and bleeding time increase predictably, and coagulogram parameters are altered. The pathology can manifest as both a quantitative shortage of blood platelets (thrombocytopenia) and their functional inadequacy (thromrasthenia).
• Management tactics: Treatment is carried out strictly in conjunction with hematologists. It is important to consider that the use of high doses of dexamethasone during therapy can lead to temporary amenorrhea.

Spontaneous hemorrhages in von Willebrand disease

Von Willebrand disease (synonym — angiohemophilia) is a hereditary coagulopathy with a mixed (bruising-hematomatous) type of bleeding. The disease affects individuals of both sexes. There are several types of genetic inheritance: type I is transmitted in an autosomal dominant manner, while types II and III are autosomal recessive (most severe clinical course).

The root cause lies in impaired synthesis of the factor VIII-plasma protein complex (von Willebrand factor), which is normally produced by endothelial cells and megakaryocytes. This factor performs a dual function:

1. In plasma hemostasis: acts as a carrier protein, ensuring the stability of coagulation factor VIII in the bloodstream.
2. In vascular-platelet hemostasis: serves as a linking element for platelet adhesion to the subendothelium in the area of vascular injury and promotes their aggregation.

• Clinical manifestations: Spontaneous hemorrhages from mucous membranes (nasal, gingival, gastrointestinal) as well as cutaneous hemorrhages (ecchymoses) are characteristic. In severe forms, the first signs manifest as early as the first year of life. Any surgical interventions (even tooth extraction or tonsillectomy) carry a high risk of massive bleeding and acute posthemorrhagic anemia.
• Differential diagnosis: Unlike classical hemophilia, in von Willebrand disease, bleeding after trauma or surgery begins immediately (there is no lucid interval), and after successful cessation, it does not recur.
• Diagnostics: Based on a burdened family history, prolonged bleeding time, decreased concentration of factors VIII and von Willebrand. A specific test shows decreased platelet aggregation in response to ristocetin.

Pharmacotherapy and management of bleeding

The approach to arresting epistaxis and other hemorrhages directly depends on the identified pathogenesis.

• Replacement therapy: In angiohemophilia, the drugs of choice are fresh frozen plasma and cryoprecipitate. Using highly purified factor VIII concentrates is inappropriate, as von Willebrand factor itself is completely absent in them.
• Drug-induced hemostasis: To stop bleeding, a synthetic analog of the antidiuretic hormone — desmopressin, as well as aminocaproic acid, are used. Important: aminocaproic acid is strictly contraindicated in renal bleeding.
• Vitamin K preparations: For prolonged epistaxis, as well as hemorrhages against the background of peptic ulcer disease, hepatitis, or obstructive jaundice, menadione sodium bisulfite (menadione) is indicated. However, it must be remembered that menadione is only a precursor of vitamin K₂. Its action develops very slowly, so the drug is absolutely ineffective for treating acute bleeding (e.g., in case of anticoagulant overdose). In emergency cases, the drug of choice is prothrombin complex concentrate, which rapidly compensates for the deficiency of factors II, VII, IX, and X.
• Social adaptation: Patients with severe coagulopathies, due to limitations in physical labor, must be instilled with an interest in intellectual activities from early childhood.

Class and exam questions

What else to study on this topic

• Mechanism of action and indications for the use of prothrombin complex concentrate in acute bleeding.
• Differential diagnosis of uterine bleeding and methods of its two-stage treatment (hemostasis and prevention).
• The role of vitamin K in the body, causes of impaired absorption, and the development of hemorrhagic disease of the newborn.
• Pathogenesis and clinical picture of indirect anticoagulant overdose (warfarin, acenocoumarol).
• Use of tocopherol (vitamin E) preparations in hematological, muscular, and reproductive disorders.

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