The Effect of Hydroxyurea on Extramedullary Hematopoietic Masses in Patients with Beta Thalassaemia Intermediate by MRI

"nIntroduction: Since the need for transfusion in thalassemia intermediate (TI) patients is limited into some rare occasions, they are almost always referred complaining of problems due to chronic anemia, bone marrow hyper functioning (such as gallstones, osteoporosis) and also extramedullary hematopoietic masses (EMH). Such masses are frequently seen around the spinal column or inside the spinal canal. Different treatment modalities have been reported for managing the latter complication. We carried out this study to evaluate the effects of Hydroxyurea in reducing the size of such masses. All patients were evaluated by MRI and the radiological findings were considered as a major criterion of response to treatment. "nMaterials and Methods: The study was a clinical trial comparing before and after characteristics of the patients referred to the thalassemia research center from 2003 to 2006. Presentation of all was pain, weakness, paresthesia of legs and paraparesis/paraplegia. Diagnosis of EHM was confirmed by spinal MRI (a 1.5 Tesla GE machine, USA). T1 and T2 weighted images with fat suppression were obtained in sagittal and axial planes of thoracic and lumbosacral spine in all patients and hematopoietic masses (more frequent in the dorsal region) were measured. Hydroxyurea (Syrea,Medac,Germany) was administered 10-20mg/kg/day. CBC,Urea,Creatinin were checked monthly. The second MRI was performed 3-6 months later. Another radiologist unaware of the sequence of imaging reported the changes of EHM during the period. Paired t test and McNemara test were applied to compare the results. P<0.05 was considered as statistically significant. "nResults: Eight patients (7 males and 1 female) were enrolled in our study. Their age ranged from 26-44 years. None had undergone transfusion, nor had they used pain killers. Neurological symptoms were initiated from one week to a couple of months prior to their admission. They had administered hydroxyurea in recommended dosage. The patients were followed up for 0.7-4.7 years. All subjects improved within 2 weeks, whereas none of them needed surgical procedures and radiotherapy. In one patient complaining of paraplegia who had large lumbar and sacral para spinal masses, the size of the lesions was greatly reduced. In all other patients, a 10 -20 percent reduction in the volume of masses was estimated. None of the subjects showed growth of the lesions in MRI. Both MCV and NRBC changes were significant (P<0.02). No considerable side effects were noticed within the follow-up period. "nConclusion: HU is proved to be both effec