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      Long segment spinal epidural extramedullary hematopoiesis

      case-report

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          Abstract

          Background:

          Extramedullary hematopoiesis is defined as the formation of blood cells outside the bone marrow. It is a common manifestation of many chronic hemolytic anemias, and typically involves the liver, spleen, and lymph nodes. Only rarely is the spinal epidural space involved.

          Methods:

          We describe a 25-year-old male, known to have thalassemia intermedia, who presented with a 1-month history of stiffness and weakness in both lower extremities. On physical examination, he had palpable splenomegaly accompanied by spinal tenderness at the D5 level, weakness in both lower extremities, hyperactive bilateral Patellar and Achilles reflexes with bilateral Babinski responses, and a graded sensory loss to pin appreciation below D5.

          Results:

          The magnetic resonance (MR) study revealed a posterior, isointense and soft tissue epidural mass extending from D2 to D12 on both the T1- and T2-weighted images. These findings were consistent with the diagnosis of “red marrow,” and long-segment spinal epidural extramedullary hematopoiesis.

          Conclusions:

          Although extramedullary hematopoiesis is rarely encountered within the spinal canal, it should be considered among the differential diagnoses when a posterior compressive thoracic lesion contributes to myelopathy in a patient with a history of thalassemia intermedia and the accompanying chronic hemolytic anemia.

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          Most cited references29

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          Paraspinal extramedullary hematopoiesis in patients with thalassemia intermedia.

          Ineffective erythropoiesis in patients with thalassemia intermedia drives extramedullary hematopoietic tumor formation in several parts of the body. Paraspinal involvement has received increasing attention due to the associated morbidity secondary to spinal cord compression. Although the history and physical examination may help narrow the differential diagnosis, radiographic imaging remains essential to confirm the existence of hematopoietic tissue. Characteristic appearance has been observed mainly on magnetic resonance imaging. Several treatment options have been described, including transfusion therapy, laminectomy, radiotherapy, and the use of fetal hemoglobin inducing agents that decrease the hematopoietic drive. However, the ideal management scheme remains controversial. Until large prospective trials evaluate the efficacy and safety of the available treatment options, both in single and in combination therapy, an individualized approach should be entertained.
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            Spinal cord compression in beta-thalassemia: case report and review of the literature.

            A case report of thoracic spinal cord compression in a 34-year-old male with beta-thalassemia is reported. In patients with thalassemia, neurologic complaints should lead to a high index of suspicion for spinal cord compression from marrow expansion, ectopic bone formation and resultant stenosis. Initial presentation, diagnosis, radiographic findings, surgical treatment and follow-up are reviewed. This case is reported from Chicago, Illinois. A chart review is performed for the purposes of this case report. Patient underwent decompressive laminectomy with good surgical outcome. Rapid diagnosis and treatment of such a condition is essential to optimize the chances of recovery.
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              Extramedullary haemopoiesis in thalassemia intermedia presenting as paraplegia.

              Extramedullary haemopoiesis causing spinal cord compression is a rare manifestation of thalassemia. We describe a 17 year old male with thalassemia intermedia who presented with progressive paraplegia and sphincter disturbance. Magnetic resonance imaging revealed an epidural lesion extending from T5 to T8 compressing the spinal cord. The patient recovered completely after surgical decompression with postoperative radiation therapy. Histological examination of the lesion confirmed the diagnosis of extramedullary haemopoiesis. Clinical awareness of this phenomenon with early treatment is essential for optimizing the neurological outcome. Copyright 2002 Published by Elsevier Science Ltd.
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                Author and article information

                Journal
                Surg Neurol Int
                Surg Neurol Int
                SNI
                Surgical Neurology International
                Medknow Publications & Media Pvt Ltd (India )
                2229-5097
                2152-7806
                2013
                26 December 2013
                : 4
                : 161
                Affiliations
                [1]Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India
                Author notes
                [* ]Corresponding author
                Article
                SNI-4-161
                10.4103/2152-7806.123657
                3883269
                24404404
                8087987c-4265-438f-a456-1351ffff8c3a
                Copyright: © 2013 Garg K.

                This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 08 October 2013
                : 11 November 2013
                Categories
                Technical Note

                Surgery
                extramedullary hematopoiesis,thalassemia,epidural
                Surgery
                extramedullary hematopoiesis, thalassemia, epidural

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