Protein C and Anti-Thrombin-III Deficiency in Children With Beta-Thalassemia

Abstract

Background: Thromboembolic events (TEEs) are recently described complications in thalassemia patients. Many mechanisms were postulated for thrombosis. Conflicting results of natural anticoagulants values were reported in previous studies. Our aim was to investigate protein C and anti-thrombin-III (AT-III) levels in thalassemics and to detect risk factors for their decrement. Methods: A cross-sectional study for 60 beta-thalassemia patients (35 major and 25 intermedia) and 35 healthy children were tested for protein C and AT-III levels, liver function tests and Sr. ferritin. Results: A significant reduction in protein C and AT-III levels was noticed in patient group compared to healthy children (82.50% (32-175) vs. 104% (60-204), P = 0.041 and 237.52 ± 53.19 mg/L vs. 322.99 ± 56.57 mg/L, P value ≤ 0.001, respectively). Protein C was lower among older patients (> 10 years) than younger patients (< 10 years), and splenectomized category than non-splenectomized one (P = 0.02 and 0.011, respectively). AT-III was significantly lower among splenectomized patients as compared to those who did not undergo splenectomy (P = 0.04). Significant correlations were found between protein C and AT-III with older age and liver functions. Conclusions: Protein C and AT-III were significantly lower among thalassemics with the main risk factors for their deficiencies being: splenectomy and increasing age. This allows establishment of early prophylactic policy against TEE for the vulnerable group.

Two cases of venous thromboembolism in siblings after splenectomy due to a novel PROC gene mutation

“…This surgery may be a predisposing factor for VTE formation because of procoagulants and abnormal platelets present on the surface of red blood cells [ 12 ]. These platelets do not disappear after splenectomy and get removed from circulation, leading to increased PC consumption to control the hypercoagulable state [ 14 – 16 ].…”