[THROMBOTIC COMPLICATIONS AND LATENT HYPERCOAGULATION IN PATIENTS WITH BETA-THALASSEMIA].

Area of Science:

• Hematology
• Vascular Medicine
• Genetics

Background:

• Beta-thalassemia (β-T) is a group of inherited blood disorders characterized by reduced or absent synthesis of beta-globin chains.
• Patients with beta-thalassemia are at an increased risk of thrombotic complications (TC) and hypercoagulable states.
• Understanding the clinical manifestations and risk factors for thrombosis in beta-thalassemia is crucial for patient management.

Purpose of the Study:

• To investigate the clinical symptoms of thrombotic complications (TC) in female patients with beta-thalassemia (β-T).
• To assess the tendency for increased thrombotic formation in patients with various forms of β-T.
• To analyze risk factors for latent hypercoagulation in β-T patients without TC or splenectomy.

Main Methods:

• A study involving 130 beta-thalassemia major (β-TM), 95 beta-thalassemia intermediate (β-TI), and 60 beta-thalassemia minor (β-Tm) patients (females, 18-40 years).
• Clinical assessment of TC, including arterial and venous thrombosis, and chronic venous insufficiency.
• Hemostasis parameters (D-dimer, fibrinogen, fibrinolytic activity, aPTT, antithrombin III) and a questionnaire on thrombosis tendency (TIT) were analyzed.

Main Results:

• TC occurred in 10.0±2.6% of β-TM and 14.7±3.6% of β-TI patients.
• TC frequency was higher in splenectomized patients (β-TM: 15.0±4.6%; β-TI: 22.5±6.6%) compared to non-splenectomized (β-TM: 5.7±2.8%; β-TI: 9.1±3.9%).
• Latent hypercoagulability (D-dimer >500 ng/ml) was found in 37.9% of β-TM and 30.4±6.8% of β-TI patients, associated with elevated D-dimer, fibrinogen, and reduced fibrinolytic activity.

Conclusions:

• Beta-thalassemia patients, particularly those with major and intermediate forms, exhibit a significant predisposition to thrombotic complications.
• Splenectomy is associated with a higher incidence of TC in β-T patients.
• A substantial proportion of β-T patients demonstrate latent hypercoagulability, highlighting the need for proactive thrombotic risk assessment and management.