Unraveling a Rare Case of Epidural Extramedullary Hematopoiesis in a Patient With Transfusion-Dependent Beta Thalassemia Presenting With Spinal Cord Compression.

Autor: Masalma R; Faculty of Medicine and Health Sciences, An-Najah National University, Nablus, PSE., Zidan T; Faculty of Medicine and Health Sciences, An-Najah National University, Nablus, PSE., Abualhumos KM; Faculty of Medicine and Health Sciences, An-Najah National University, Nablus, PSE., Hamayel D; Faculty of Medicine and Health Sciences, An-Najah National University, Nablus, PSE., Abukhalil Z; Department of Neurosurgery, Palestine Medical Complex, Ramallah, PSE., Ghanem AT; Department of Neurosurgery, Palestine Medical Complex, Ramallah, PSE., Mousa A; Faculty of Medicine and Health Sciences, An-Najah National University, Nablus, PSE., Helou M; Faculty of Medicine and Health Sciences, An-Najah National University, Nablus, PSE., Tamimi W; Faculty of Medicine and Health Sciences, An-Najah National University, Nablus, PSE., Al-Sayed Ahmad M; Faculty of Medicine and Health Sciences, An-Najah National University, Nablus, PSE.
Jazyk: angličtina
Zdroj: Cureus [Cureus] 2024 Mar 17; Vol. 16 (3), pp. e56352. Date of Electronic Publication: 2024 Mar 17 (Print Publication: 2024).
DOI: 10.7759/cureus.56352
Abstrakt: Thalassemia is known to induce extramedullary hematopoiesis (EMH), which is a compensatory mechanism in which the body forms blood cells outside the bone marrow. While EMH typically affects organs such as the spleen and liver, there are rare instances where it leads to spinal cord compression (SCC) in the epidural space. A 31-year-old male patient with transfusion-dependent beta thalassemia presented with numbness and bilateral limb weakness due to EMH. Neurological examination revealed increased tone in both legs, reduced power, loss of crude touch and pain sensation, and increased deep tendon reflexes. Magnetic resonance imaging (MRI) indicated a lobulated soft tissue structure in the posterior dural intrathecal space causing SCC. Laminectomy of the T2-T8 vertebrae was done, after which the lesion was identified and completely removed. Post-surgery, significant neurological improvements were observed in both motor and sensory functions. Thalassemia patients presenting with symptoms of SCC should be investigated for the presence of epidural EMH. Treatment options include decompressive surgery, blood transfusions, hydroxyurea, and radiotherapy.
Competing Interests: The authors have declared that no competing interests exist.
(Copyright © 2024, Masalma et al.)
Databáze: MEDLINE