Coagulation Defects in Hemoglobin E β Thalassemia Patients: A Study from a Thalassemia Care Centre (TCU) in Kolkata,...

Thalassemia is known to be a hypercoagulable state but limited literature is available on coagulation defects in HbE β-thalassemia. The present work aimed to study the coagulation defects in HbE β-thalassemia.Total 80 cases of HbE β-thalassemia and 40 cases of healthy controls were included. Prothrombin time (PT), Activated partial thromboplastin time (aPTT), fibrinogen, protein C, protein S and antithrombin levels were compared between thalassemia patients and controls, and TDT and NTDT patients. Correlation of serum ferritin with coagulation parameters was studied. 50% of the patients were TDT and 50% were NTDT. Only 7.5% patients were splenectomized. Out of total 80 patients, 32.5% had prolonged PT (≥ 15 s), and 17.5% had prolonged aPTT (≥ 39.8 s), whereas, only 7.5% and 2.5% in the control group (n = 40) had prolonged PT and aPTT respectively. The mean PT and aPTT of the TDT, NTDT and total patients were significantly more compared to the mean PT of controls. However, there was no significant difference in mean PT and mean aPTT between TDT vs. NTDT and splenectomized vs. non splenectomized patients, respectively. Fibrinogen was low (< 150 mg/dl) in 11.25% of the patients (n = 80), 73.75% had low Protein C (< 70%), 73.75% had low Protein S (< 60%) and 53.75% had low antithrombin (< 79.4%). The mean fibrinogen, protein C, protein S and antithrombin levels of TDT, NTDT and the total patients were significantly low compared to that of controls. However, there was no significant difference in fibrinogen, protein C and antithrombin levels between TDT vs. NTDT and splenectomized vs. non splenectomized patients. Whereas, Protein S levels of splenectomized patients was significantly low compared to non splenectomized patients with no difference between TDT and NTDT. No association was seen between serum ferritin with any of the studied coagulation parameters (p > 0.05).Hence, from the study, we conclude that HbE β-thalassemia is a hypercoagulable state irrespective of transfusion dependence and iron overload status.