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Case Reports
. 2019 Oct 17:2019:5827626.
doi: 10.1155/2019/5827626. eCollection 2019.

Compressive Dorsal Myelopathy Secondary to Extramedullary Hematopoiesis in a Thalassemic Patient

Ismail Ibrahim Ismail  1 , Fathi Massoud  1 , K J Alexander  1 , Jasem Youssef Al-Hashel  1   2
Affiliations
Case Reports

Compressive Dorsal Myelopathy Secondary to Extramedullary Hematopoiesis in a Thalassemic Patient

Ismail Ibrahim Ismail et al. Case Rep Neurol Med. .

Abstract

Background: Extramedullary hematopoiesis (EMH) is one of the rare causes of spinal cord compression (SCC). It results from noncancerous proliferation of hematopoietic tissue outside the bone marrow as a compensatory mechanism for ineffective erythropoiesis. It occurs in the paraspinal area in 11-15% of thalassemic patients in intermediate and severe cases causing a paraspinal compressive mass. We present a rare case of spinal EMH with thoracic cord compression in a 22-year-old female with beta thalassemia who presented with paraparesis and we provide a review of literature.

Case report: A 22-year-old female patient with a known history of beta thalassemia presented with subacute onset of weakness and numbness of both lower limbs with a sensory level at T6. Magnetic resonance imaging (MRI) of the dorsal spine showed cord compression secondary to paraspinal EMH from T2 to T9 with most prominent compression over T5. She was managed with blood transfusion and low-dose radiotherapy.

Conclusion: Although rare, EMH should be suspected in thalassemic patients presenting with paraplegia. Treatment with blood transfusions is usually effective. Other options include radiotherapy, surgery, hydroxyurea or a combination of these modalities.

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Conflict of interest statement

The authors declare that they have no conflicts of interest.

Figures

Figure 1
Figure 1
MRI of the dorsal spine: (a) sagittal T2-weighted, (b) axial T1-weighted images with contrast showing posterior epidural multilobulated extramedullary hematopoietic mass from T2 to T9 (arrows) with the largest at T5 causing the most prominent cord compression and showing no contrast enhancement.
Figure 2
Figure 2
Follow up MRI of the spine after blood transfusion: (a) sagittal T2-weighted, (b) sagittal T1-weighted, (c) coronal T1-weighted images showing interval reduction in cord compression.
See this image and copyright information in PMC

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