Spinal Cord Compression in Homozygous Beta-Thalassemia Intermedia

Cardia, E.; Toscano, Samantha; Rosa, G. La; Zaccone, C.; D’Avella, Domenico; Tomasello, Francesco

doi:10.1159/000120785

Cited by 18 publications

(12 citation statements)

References 12 publications

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“…A commonplace vascular territory proposition has also been introduced accordingly, relating the thoracic predilection to development of EMH from branches of the intercostal veins 13 , or metameric arterial embolic phenomena 14 . EMH usually forms a soft deep red mass which resembles a hematoma on its cut surface.…”

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Progressive Spinal Cord Compression Due to Epidural Extramedullary Hematopoiesis in Thalassemia Intermedia

2013

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Extramedullary hematopoiesis (EMH) can occur in patients with thalassemia intermedia as a physiologic response to insufficient erythropoiesis to meet circulatory demands. Rarely, EMH involve the epidural space causing spinal cord compression. We describe a case of EMH in a 21-year-old man with β-thalassemia intermedia presenting with progressive low back pain, worsening paraparesis and sphincter disturbance. Magnetic resonance imaging (MRI) demonstrated spinal cord compression by EMH epidural mass lesions extending from T3 to T10 levels. An emergency multilevel decompressive laminectomy was performed with excision of the masses and complete rehabilitation of the patient.

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Progressive Spinal Cord Compression Due to Epidural Extramedullary Hematopoiesis in Thalassemia Intermedia

2013

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“…However, it is understood that >80% of cases do not have signs and symptoms related directly to the disorder, and the lesions are typically discovered incidentally by radiologic techniques (15–17). The development of neurologic symptoms is suggested to depend on the chronicity of the disease, with neurologic symptoms most frequently being reported during the third and fourth decades of life (18), although few reports described presentation as early as the first decade of life (19–21). The male to female ratio reaches 5:1 (18).…”

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confidence: 99%

Extramedullary hematopoiesis: A report of two cases

Zhang

Liu

et al. 2016

Experimental and Therapeutic Medicine

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Extramedullary hematopoiesis (EMH) is defined as hematopoiesis occurring in organs outside of the bone marrow. The present report describes two cases of thalassemic patients with paraspinal medullary hematopoiesis and analyzes the clinical manifestations, imaging, pathology, diagnosis and treatment of EMH. In addition, a supplementary review of previously published cases is provided along with a review of the related literature. Computed tomography (CT) of the first case revealed multiple paraspinal masses, and the largest was 6.2×8.0 cm in diameter. Likewise, CT of the second patient revealed multiple paraspinal masses in the bottom of the left thoracic cavity, and the largest was measured 10.1×10.5 cm. The two cases underwent surgical biopsy and the findings were compatible with a diagnosis of EMH. In conclusion, EMH is a compatible and rare disease, and should be distinguished from other neoplasms. EMH must considered when masses with characteristic radiologic appearance are detected in patients with thalassemia intermedia.

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“…However this therapy has its own complications associated with transfusion and the response is slow, temporary and insufficient. 10 Low dose radiation has been reported to yield excellent result in upto 50% of patients, with neurological International Journal of Spine Surgeryimprovement seen in 3-7 days of initiation.…”

Section: Discussionmentioning

confidence: 99%