Maiti Abhishek, Chakraborti Amartya, Chakraborty Puranjoy, Mishra Sanjay
West Bank Hospital.
Australas Med J. 2012;5(2):152-5. doi: 10.4066/AMJ.20121076. Epub 2012 Feb 29.
Alterations of coagulation profile have been reported in patients with β-thalassaemia major (β-TM).
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.
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 liner correlation could be identified between PT, APTT, PC of the patients and interval between transfusions (in days) or days since last transfusion.
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.
已有报道称重型β地中海贫血（β-TM）患者的凝血谱发生改变。
为了在儿科人群中对此进行研究，我们对50名未行脾切除术的输血依赖型β-TM儿童（平均年龄6±2.5岁）输血前血样中的止血参数以及25名健康对照者的血液进行了研究。
实验室评估显示，40%的患者存在血小板减少，12%的患者凝血酶原时间（PT）延长，6%的患者活化部分凝血活酶时间（APTT）延长。与对照组相比，患者组的PT、APTT和血小板计数（PC）平均值均升高。凝血状态的改变在PT方面具有显著性（p值<0.005），在APTT方面也具有显著性（p值<0.0001）。然而，PC的变化不具有显著性（p值<0.05）。患者的PT、APTT、PC与输血间隔天数（以天计）或上次输血后的天数之间未发现显著的线性相关性。
本研究结果表明，β-TM患者在很小的年龄就存在亚临床出血倾向。内源性途径似乎比外源性途径受影响更大。
