Lumbar nerve root compression due to extramedullary hemopoiesis in a patient with thalassemia: complete clinical regression with radiation therapy. Case report and review of the literature.

The authors describe the case of a 24-year-old woman who had a history of beta3-thalassemia and presented with severe symptoms of lumbar nerve root compression due to extramedullary hemopoiesis in the intervertebral foramen. Radiation therapy (2000 cGy in six fractions) was delivered to the mass. The patient's neurological symptoms completely resolved following treatment. Follow-up images showed a reduction in lesion size. The results of this unique case supported by a review of the literature suggest that radiation therapy alone is an effective modality in the treatment of patients with compression of neural structures due to extramedullary hemopoietic tissue.

Radiotherapy combined with erythropoietin for the treatment of extramedullary hematopoiesis in an alloimmunized patient with thalassemia intermedia.

We report a patient with thalassemia intermedia who developed a mediastinal syndrome due to the growth of paravertebral hematopoietic masses in the posterior mediastinum. Because the patient did not receive blood transfusions due to alloimmunization, she was first treated with human recombinant erythropoietin (escalating low-moderate doses) to recover hemoglobin levels, then in association with radiotherapy to prevent a worsening of her anemia. The mean Hb level dramatically increased and peaked at week 11, to 83 g/l, and remained unchanged before and after radiotherapy (81 versus 78 g/l). Immediately after radiotherapy extramedullary hematopoiesis volume decreased by 16.4%.

The role of radiation therapy in the management of spinal cord compression due to extramedullary haematopoiesis in thalassaemia.

Extramedullary haematopoiesis associated with thalassaemia leading to spinal cord compression is an extremely rare event in the course of the disease. The efficacy of radiation therapy is advocated in the management of such a complication. Two patients with thalassaemia, who had presented with spinal cord compression, were successfully treated by a modest dose of local radiotherapy. In one of the patients, however, radiotherapy was resorted to after an initial decompressive laminectomy and partial removal of the intraspinal haematopoietic mass proved unsuccessful. The other patient was managed solely by radiation therapy.

The role of radiation therapy in the management of hematopoietic neurologic complications in thalassemia.

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.

Spinal cord compression in thalassemia. Report of 12 cases and recommendations for treatment.

Twelve patients with beta-thalassemia/hemoglobin E disease had spinal cord compression. Ten were made and two female, aged 17 to 40 years. The causes of spinal cord compression presumably were extramedullary hematopoietic masses. This was proved by surgery in two cases. In six cases, myelography demonstrated extradural blockade. In the others, the recurrent nature of the paraparesis and the prompt response to deep x-ray therapy were compatible with cord compression by extramedullary hematopoietic masses. Although spontaneous recovery and disappearance of the neurological signs after blood transfusions were observed, these were slow and uncertain. Deep x-ray therapy led to prompt response with more lasting benefit in all cases and is thus recommended as standard treatment for this complication.