Paraplegia due to Extramedullary Hematopoietic Tissue Compression of Thoracic Thecal Sac in a Patient with Thalassemia Treated Successfully with Surgical Decompression: A Case Report

Abstract

Spinal cord compression due to extramedullary hematopoiesis (EMH) is a rare complication of thalassemia and generally presents as paraparesis with sensory impairment. Complete paraplegia is extremely rare in EMH due to thalassemia although it is known to occur in polycythemia vera and sickle cell anemia. Cases presenting with paraparesis have been treated with either surgery or radiotherapy with equal frequency and efficacy. Almost all reported cases with paraplegia have been treated with surgery with or without radiation therapy. As paraplegia secondary to EMH is rare, there is no consensus on treatment. We hereby report a case of thalassemia with paraplegia treated successfully with surgery. Treatment options for cord compression include primarily blood transfusion, surgery, and radiation therapy. Because of the extreme rarity of this condition, direct comparisons between various treatment modalities are not possible. The bias toward surgery is due to its immediate decompressing effect. EMH in thalassemia has also been treated with transfusion therapy with the rationale that correction of anemia would downregulate erythropoietin and lead to reversal of EMH. However, improvement with blood transfusion alone is usually incomplete and slow. Our patient did not improve after adequate blood transfusion and hence underwent surgical decompression after which he showed rapid and complete neurologic recovery. Therefore, we conclude that surgical decompression of EMH tissue has to be considered early in patients presenting with paraplegia if symptom shows no improvement after adequate blood transfusion.

Keywords

extramedullary hematopoietic - decompressive laminectomy - thalassemia major

• References

• 1 Malik M, Pillai LS, Gogia N , et al. Paraplegia due to extramedullary hematopoiesis in thalassemia treated successfully with radiation therapy. J Clin Neurosci 2002; 9 (6) 721-725
• 2 Salehi SA, Koski T, Ondra SL. Spinal cord compression in beta-thalassemia: case report and review of the literature. Spinal Cord 2004; 42 (2) 117-123
• 3 Malik M, Pillai LS, Gogia N , et al. Paraplegia due to extramedullary hematopoiesis in thalassemia treated successfully with radiation therapy. Haematologica 2007; 92 (3) e28-e30
• 4 141900:haemoglobin—beta locus;HBB: http://www.omim.org/entry/141900
• 5 Gatto I, Terrana V, Biondi L. Compression on the spinal proliferation of one marrow in the epidural space in the subject suffering from disease Colley splenectomized. [Italian] Hematological 1954; 38: 61-75
• 6 Issaragrisil S, Piankigagum A, Wasi P. Spinal cord compression in thalassemia. Report of 12 cases and recommendations for treatment. Arch Intern Med 1981; 141 (8) 1033-1036
• 7 Tan TC, Tsao J, Cheung FC. Extramedullary haemopoiesis in thalassemia intermedia presenting as paraplegia. J Clin Neurosci 2002; 9 (6) 721-725
• 8 Munn RK, Kramer CA, Arnold SM. Spinal cord compression due to extramedullary hematopoiesis in beta-thalassemia intermedia. Int J Radiat Oncol Biol Phys 1998; 42 (3) 607-609
• 9 Coşkun E, Keskin A, Süzer T, Sermez Y, Kildaci T, Tahta K. Spinal cord compression secondary to extramedullary hematopoiesis in thalassemia intermedia. Eur Spine J 1998; 7 (6) 501-504