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.