Background

Alterations of coagulation profile have been reported in patients with β-thalassaemia major (β-TM).

Method

To investigate this in the paediatric population, we studied haemostatic parameters in pre-transfusion blood samples from 50 non-splenectomised transfusion-dependent children with β-TM (mean age 6±2.5 years) and in blood from 25 healthy controls.

Results

Laboratory evaluation showed thrombocytopenia in 40%, prolongation of prothrombin time (PT) in 12% and prolongation of activated partial thromboplastin time (APTT) in 6% of the patients. Mean values for PT, APTT and platelet count (PC) were all raised in the patient population compared with the controls. The alteration of coagulation status was significant for PT (p value < 0.005) and APTT (p value < 0.0001). However, the change for PC was not significant (p value > 0.05). No significant linear correlation could be identified between PT, APTT, PC of the patients and interval between transfusions (in days) or days since last transfusion.

Conclusion

The findings from this study suggest that a subclinical haemorrhagic tendency exists in patients with β-TM at a very early age. The intrinsic pathway appears to be more affected than the extrinsic pathway.