Progressive compressive thoracic myelopathy

Abstract

Extramedullary hematopoiesis is a possible complication in patients with beta-thalassemia, due to ineffective erythropoiesis, producing remote sites of hematopoiesis in multiple and very variable anatomic locations [1Gatto 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: 61-76PubMed Google Scholar, 2Haidar R. Mhaidli H. Taher A.T. Paraspinal extramedullary hematopoiesis in patients with thalassemia intermedia.Eur Spine J. 2010; 19: 871-878Crossref PubMed Scopus (126) Google Scholar]. Among these sites, spinal epidural extramedullary hematopoiesis carries potential risk and may lead to acute irreversible neurological deficits [[3]Salehi S.A. Koski T. Ondra S.L. Spinal cord compression in beta-thalassemia: case report and review of the literature.Spinal Cord. 2004; 42: 117-123Crossref PubMed Scopus (47) Google Scholar]. Surgery has been used as a treatment option, however there have been reports of significant blood loss [3Salehi S.A. Koski T. Ondra S.L. Spinal cord compression in beta-thalassemia: case report and review of the literature.Spinal Cord. 2004; 42: 117-123Crossref PubMed Scopus (47) Google Scholar, 4Lau S.K. Chan C.K. Chow Y.Y. Cord compression due to extramedullary hemopoiesis in a patient with thalassemia.Spine. 1994; 19: 2467-2470Crossref PubMed Scopus (35) Google Scholar, 5Garg K. Singh P.K. Singh M. et al.Long segment spinal epidural extramedullary hematopoiesis.Surg Neurol Int. 2013; 4: 161Crossref PubMed Google Scholar]. In our case, after multidisciplinary discussion, emergency surgical decompression was chosen due to the rapidly progressive paraparesis during the preceding48 hours since admission. It was felt that this would provide speedier decompression over radiotherapy. Additionally, appropriate preoperative hematologic optimization (tranexamic acid, blood and platelet transfusions) was also instigated. After induction of general anesthesia, left T5-T9 hemilaminectomies) were performed (Fig. 2). Total intraoperative blood loss was estimated at 200cc. No supplementary hematologic treatment was needed in the postoperative period. We underline the need for surgical management in cases of progressive spinal cord compression [2Haidar R. Mhaidli H. Taher A.T. Paraspinal extramedullary hematopoiesis in patients with thalassemia intermedia.Eur Spine J. 2010; 19: 871-878Crossref PubMed Scopus (126) Google Scholar, 4Lau S.K. Chan C.K. Chow Y.Y. Cord compression due to extramedullary hemopoiesis in a patient with thalassemia.Spine. 1994; 19: 2467-2470Crossref PubMed Scopus (35) Google Scholar]. It can be performed safely and without excessive hemorrhagic risk. In our case we used a unilateral approach which allowed complete decompression. This approach also limits blood loss and preserves soft tissue and bony structures on the contralateral side. Adequate preoperative hematologic status is essential in these cases. Radiation therapy was used as an adjuvant treatment postoperatively to prevent recurrence of epidural extramedullary hematopoiesis. Follow-up was performed at 6 weeks and 3 months. Complete neurologic recovery was observed, with no recurrence at 3 months MRI (Fig. 3). In conclusion, the optimal management for extramedullary hematopoiesis with neurological impairment remains unproven and is currently based on case-by-case situations. We suggest that surgical management can be safely performed with optimal hematologic preparation without incurring excessive intraoperative blood loss. Note 1: A signed patient consent form has been supplied for publication. Note 2: Posthumous authorship for Dr Christian Wider.