Introduction
Haemoglobinopathies constitute the commonest recessive monogenic disorders worldwide. They fall into two main groups: the thalassemia syndromes and the structural hemoglobin variants (abnormal hemoglobin). α, β, and δβ thalassemia are the main types of thalassemia with clinical importance; the most frequent and clinically important structural hemoglobin variants are HbS, HbE, HbC and HbD. The treatment of patients presents a substantial global disease burden. Variants of thalassemia and main abnormal hemoglobin interact to produce a wide range of clinical disorders of varying severity (1). From clinical perspective thalassemia syndromes were classified into Transfusion dependent thalassemia and non-transfusion dependent thalassemia (2).Most of the complications of thalassemia results from iron over load with liver and heart being the main cause for morbidity and mortality (3).Other complications mainly endocrine like osteoporosis (4),Diabetes (5),Thyroid (6),Hypogonadism and fertility (7)(8),As well as cortisol abnormalities(9). Patient with thalassemia should go for ALLO-SCT (Allogeneic stem cell transplantation ) if HLA identical sibling is available or to maintain transfusion and chelating therapy lifelong in the form of Desferal, Deferiprone or deferasirox or its new film coated formula .(10).
The risk of neurologic compromise in this disease is in part due to the fact that extramedullary hematopoiesis within the spinal canal can result in the compression of neural structures. There have only been a few reports in the literature describing spinal cord compression by such a disease process(11)(12)
treatment options have been described, including transfusion therapy(13)(11), laminectomy, radiotherapy(12)(14)(15) , and the use of fetal hemoglobin inducing agents that decrease the hematopoietic drive. However, the ideal management regimen remains controversial.