Spinal cord compression due to extramedullary hematopoiesis in beta-thalassemia

Compressão da medula espinhal por hematopoese extramedular na beta-talassemia

This study reports the case of a 35-year-old man with beta-thalassemia intermedia, with irregular treatment, who presented progressive paresthesia mainly affecting the left side and strength loss in the lower limbs for five months. Besides, he developed difficulty to ambulate and urinate. MRI (Figures 1, 2, 3 and 4) showed vertebral canal stenosis with spinal cord compression. This finding illustrates a rare complication of thalassemia, an extramedullary hematopoietic center due to ineffective erythropoiesis, causing vertebral bone hyperplasia^1,4,5. Given the substantial number of segments affected, surgical procedures were contraindicated based upon hemorrhage risk. Radiotherapy was performed with subsequent strength recovery ^2,3.

Figure 1.
T1-weighted axial MRI with contrast showing moderate contrast enhancement of the epidural masses. Ribs and the vertebral bodies are also enhanced.

Figure 2.
T2-weighted axial MRI showing severe spinal cord compression (the black arrows indicate the ribs and the white one indicates the vertebral bone).

Figure 3.
T1-weighted sagittal MRI showing moderate and heterogeneous enhancement of epidural masses.

Figure 4.
T2-weighted sagittal MRI showing spinal cord compression caused by an extensive epidural lesion.

References

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  Yathiraj PH, Singh A, Vidyasagar S, Varma M, Mamidipudi V. Excellent and durable response to radiotherapy in a rare case of spinal cord compression due to extra-medullary hematopoiesis in β-thalassemia intermedia: case report and clinicoradiological correlation. Ann Palliat Med. 2017 Apr;6(2):195-9. https://doi.org/10.21037/apm.2016.12.05
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Publication Dates

• Publication in this collection
  26 Oct 2020
• Date of issue
  Oct 2020

History

• Reviewed
  23 Mar 2020
• Received
  05 May 2020
• Accepted
  08 May 2020