Diagnosis And Treatment Of Cord Compression Secondary To Extramedullary Hematopoiesis In Patients With Beta-Thalassemia Intermedia
Dr. Fakher Rahim, Physiology Research Center, Ahwaz Jondishapur Univesity of Medical Sciences, Ahwaz, Iran. Email: firstname.lastname@example.org
Background: Thalassemia is an inherited autosomal recessive hematological disorder due to genetic defect in synthesis of one of the globin chains. This results in reduced rate of hemoglobin formation and presents as anemia. Depending on whether alpha or beta globin chain is affected they are classified respectively as α thalassemia and β thalassemia. If only one β globin allele bears a mutation, the disease is called β thalassemia minor and if both alleles have thalassemia mutations, the disease is called β thalassemia major. Thalassemia intermedia(TI) is a condition intermediate between the major and minor forms. Patients with TI do not receive regular blood transfusions unlike thalassemia major patients. Extramedullary hematopoiesis (EMH) is a compensatory mechanism that occurs in patients with hematological dysfunctions such as Thalassemia Major or Thalassemia Intermedia and Sickle Cell Anemia as a result of continuous erythropoietic stress.
Materials and Methods: We report two cases of TI with EMH. One is a 17 year old girl who presented with back pain and leg weakness and a 25 year old man who was referred to the hospital with back pain, paresthesia, urine frequency and impairment of gait.
Results: Both the patients were successfully treated with low dose radiotherapy and Hydroxyurea (HU). At the end of the therapy, both the patients had recovered well and were ambulatory.
Discussion: Surgical decompression has been the method of choice for the management of the disease. The disadvantages of surgical intervention include risk of excessive bleeding due to high vascularity of the mass. Low dose radiotherapy and hydroxyurea offer better outcomes with reduced morbidity