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.
Highlights
The thalassemia is congenital quantitative defect in hemoglobin synthesis
The first documented cases of spinal cord compression from Extramedullary haematopoiesis (EMH) was described by Gatto in 1954, based on clinical examination and myelography.11Treatment of spinal cord compression due to EMH may be radiation therapy to halt the production of overgrown marrow tissue, surgical decompression or a combination of both.[2, 12]
We present here a case of Thalassemia with spinal cord compression due to extramedullary Haematopoesis in a twenty one year old female whose neurological symptoms recovered near completely with External beam radiotherapy
Summary
The thalassemia is congenital quantitative defect in hemoglobin synthesis. It produces normal hemoglobin but in reduced amounts.[1]. Most authors do not favor a tissue biopsy in this situation.[9] The onset of neurologic symptoms in a known patient of thalassemia should raise concern for cord or thecal sac compression by an EMH.[10] The first documented cases of spinal cord compression from EMH was described by Gatto in 1954, based on clinical examination and myelography.11Treatment of spinal cord compression due to EMH may be radiation therapy to halt the production of overgrown marrow tissue, surgical decompression or a combination of both.[2, 12]
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