The Role of Radiation Therapy in the Management of Hematopoietic Neurologic Complications in Thalassemia

Abstract

Extramedullary hematopoiesis is encountered in some severe anemic conditions. Very rarely it may cause neurologic symptoms, leading to spinal cord or cauda equina compression. Two patients with thalassemia who developed neurologic complications are described. Diagnosis was based on clinical findings, CT scan and magnetic-resonance imaging (MRI). The two patients responded successfully to irradiation and blood transfusion. The prognosis is excellent, if recognized at an early stage. Our experience supports the role of radiation therapy as standard treatment for this complication.

Painful Spinal Cord Compression as a Complication of Extramedullary Hematopoiesis Associated with β-Thalassemia Intermedia

“…But as in our case, the reduction of hematopoietic pseudotumors happens slowly and incomplete [6,12]. In patients who need rapid therapeutic response due to severe neurological symptoms, radiotherapy might be considered the primary treatment.…”

Regression of extramedullary hematopoiesis with hydroxyurea therapy in ß-thalassemia intermedia

“…6 HU has been associated with increase of the total percentage of fetal hemoglobin and the number of F-cells, as well as the total hemoglobin concentration and mean corpuscular volume in sickle cell patients. 7 Although there is limited experience with HU in thalassemia, some studies have demonstrated successful regression of EMH with HU therapy.…”

Spinal cord compression in beta-thalassemia: case report and review of the literature

“…The benefits include ready availability, effectiveness in the resolution of symptoms in a short period of time and reduction of local recurrence. 17 Abassioun and Amir-Jamshidi 8 in 1982 reported the case of a 15-year-old female who was paraparetic with long tract signs. The patient's symptoms resolved after 1500 cGy of radiation in five treatments with only residual right sustained clonus.…”