Email this article 
Post a Comment 
Thoracic spinal cord compression due to xtramedullary haemopoiesis in a patient with beta-thalassemia: complete clinical regression with radiation therapy alone
Abstract
Spinal cord compression due to Extramedullary Haemopoesis in beta-thalassemia is extremely rare. Controversies are there between the two modalities of treatment surgery vs radiation therapy. We present here a case of beta thalassemia major in a twenty one years female patient who presented with features of spinal cord compression due to extramedullary haemopoesis.. She was then treated with 3000 cGy of radiation therapy targeted to the T5-T8 region, as 200 cGy/fraction daily, 5 fractions/week , over 6 weeks .The patient’s haemoglobin was elevated from 6.1g/dl to 10.1g/dl, with her haematocrit rising from 26.3 % to 32.8%.Steroid dose was tapered on hospital day number 7.She achieved near full neurological recovery after medical treatment with steroids, blood transfusion and radiation therapy.
Keywords
Full Text:
PDFReferences
Wintrobe MM, Greer JP. Wintrobe's Clinical hematology. 11th ed. Philadelphia: Lippincott Williams & Wilkins; 2004.
Issargisil S, Piankijagum A, Wasi P. Spinal cord compression in thalassemia. Report of 12 cases and recommendations for treatment. Arch Intern Med 1981; 141: 1033-6.
Lichtman MA, Williams WJ. Williams Hematology. 7th ed. New York: McGraw-Hill Medical Pub. Division; 2006.
Meo A, Cassinerio E, Castelli R, et al. Effect of hydroxyurea on extramedullary haematopoiesis in thalassaemia intermedia: case reports and literature review. Int J Lab Hematol 2008; 30: 425-31.
Prabhakar S, Chopra JS, Khosla VK, et al. Spinal cord compression in homozygous beta thalassemia. Surg Neurol 1980, 13: 351-4.
Chehal A, Aoun E, Koussa S, et al. Hypertransfusion: a successful method of treatment in thalassemia intermedia patients with spinal cord compression secondary to extramedullary hematopoiesis. Spine 2003; 28: E245-9.
Khen-Dunlop N, Girot R, Brunelle F, et al. Surgical treatment of an unusual case of pelvic extramedullary hematopoiesis. J Pediatr Surg 2006; 41:e13-5.
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: 607-9.
Kaufmann T, Coleman M, Giardina P, Nisce LZ. The role of radiation therapy in the management of hematopoietic neurologic complications in thalassemia. Acta Haematol 1991; 85: 156-9.
Salehi SA, Koski T, Ondra SL. Spinal cord compression in beta-thalassemia: case report and review of the literature. Spinal Cord 2004; 42: 117-23.
Gatto I, Terrana V, Biondi L. Compression of the spinal cord due to proliferation of bone marrow in epidural space in a splenectomized person with Cooley's disease. Haematologica 1954; 38:61-75.
Lau SK, Chan CK, Chow YY. Cord compression due to extramedullary haemopoiesis in a patient with thalassemia. Spine 1994; 19: 2467-70.
Singhal S, Sharma S, Dixit S, et al. The role of radiation therapy in the management of spinal cord compression due to extramedullary hematopoiesis in thalassemia. J Neurol Neurosurg Psychiatry 1992; 55: 310-2.
Jackson DV Jr, Randall ME, Richards F. Spinal cord compression due to extramedullary hematopoiesis in thalassemia: long term follow up after radiotherapy. Surg Neurol 1988; 29:388-92.
Niggemann P, Krings T, Hans F, Thron A. Fifteen-year follow-up of a patient with beta thalassaemia and extramedullary haematopoietic tissue compressing the spinal cord. Neuroradiology 2005; 47: 263-6.
Papavasiliou C, Sandilos P. Effect of radiotherapy on symptoms due to heterotopic marrow in beta-thalassaemia. Lancet 1987; 1: 389-90.
Tan TC, Tsao J, Cheung FC. Extramedullary haemopoiesis in thalassemia intermedia presenting as paraplegia. J Clin Neurosci 2002; 9: 721-5.
DOI: http://dx.doi.org/10.14319/ijcto.0301.7
This work is licensed under a Creative Commons Attribution 3.0 License.
International Journal of Cancer Therapy and Oncology (ISSN 2330-4049)
© International Journal of Cancer Therapy and Oncology (IJCTO)
To make sure that you can receive messages from us, please add the 'ijcto.org' domain to your e-mail 'safe list'. If you do not receive e-mail in your 'inbox', check your 'bulk mail' or 'junk mail' folders.
Number of visits since October, 2013