Objective: Hypercoagulation in β-thalassemia patients is known to manifest as arterial and/or venous thrombotic complications. Along with the clinical assessment of thrombotic complications (TC), it is also important to study latent (masked) hypercoagulation (LH) hypercoagulable state (HS) in patients with β-thalassemia. HS assessment is possible based on the analysis of various clinical symptoms and patient history.
Case report: In the National Centers of Hematology and Transfusiology, we studied 315 women aged 18–40 years: 130 with β-thalassemia Major (TM), 95 with β-thalassemia intermedia (TI), 60 with β-thalassemia minor (Tm), 30 blood donors (BD).
Methodology: The data were analyzed retrospectively and as a result of our survey on the increased thrombotic tendency. Statistics: data input system MS Excel, data processing using the program Statistics 6.
Results: In 10.0±2.6% of TM patients and in 14.7±3.6% of TI patients, various TCs were revealed: arterial thrombosis, venous thrombosis, chronic venous insufficiency (varicose nodes of the lower extremities, telangiectasia, trophic ulcer, venous eczema, swelling of the feet and lower legs). Such complications was not detected in patients with Tm and in the control group. Out of 60 splenectomized patients with TM, arterial thrombosis was observed in 2 (3.3%) patients, venous thrombosis in 3 (5.0%) patients, and signs of chronic venous insufficiency in 4 (6.7%) patients. Out of 70 non-splenectomized patients with TM, venous thrombosis was observed in 1 (1.4%) patient, and signs of chronic venous insufficiency in 3 (4.3%) patients. Of the 40 splenectomized TI patients, arterial thrombosis was observed in 2 (5.0%), venous thrombosis in 3 (7.5%), and signs of chronic venous insufficiency in 4 (10%). Of 55 non-splenectomized TI patients, venous thrombosis was observed in 2 (3.6%), and signs of chronic venous insufficiency in 3 (5.4%). Assessment of thrombotic tendency was conducted among non-splenectomized patients. HS (the total score for the PTT questionnaire >30) was detected in 36.0±6.8% of TM patients and 40.0±7.7% of TI patients. In patients with TI and in BD, increased thrombotic tendency was not detected (the sum of the scores for the PTT questionnaire is <30).
Conclusion: TCs detected in patients with homozygous β-thalassemia was more common in patients with TI compared with patients with TM (p≥0.05). In patients, cases of venous thrombosis were detected 2 times more often than arterial thrombosis (p≥0.05). Chronic venous insufficiency was detected identically in patients with TM and TI. TCs was observed more often in splenectomized patients with TM and TI compared with non-splenectomized patients (p≥0.05). It was established that some patients with β-thalassemia who did not have clinical thrombotic complications had prethrombotic state. A study of clinical and anamnestic risk factors revealed a tendency to HS in 1/3 of patients with β-thalassemia. Based on the results of the survey, the risk factors (predictors) of HS were determined. The tendency to form blood clots in patients with anemia was associated with two groups of clinical and anamnestic symptoms: “comorbidity” and “chronic stress conditions”.
