Progressive Spinal Cord Compression Due to Epidural Extramedullary Hematopoiesis in Thalassemia Intermedia: A Case Report and Literature Review

The Neuroradiology Journal, 2013 · DOI: · Published: January 1, 2013

Simple Explanation

Extramedullary hematopoiesis (EMH) is when blood cell production occurs outside the bone marrow, often as a response to the body's need for more blood cells. In patients with thalassemia intermedia, EMH can lead to masses forming outside the bones, and in rare cases, these masses can compress the spinal cord. This paper describes a case where a man with thalassemia intermedia experienced progressive spinal cord compression due to EMH, requiring surgical intervention.

Study Duration

Not specified

Participants

One 21-year-old man with β-thalassemia intermedia

Evidence Level

Case Report and Literature Review

Key Findings

1
MRI demonstrated spinal cord compression by EMH epidural mass lesions extending from T3 to T10 levels.
2
The lesions displayed intermediate signal intensity on T1- and T2-weighted images with mild enhancement in post contrast images.
3
An emergency multilevel decompressive laminectomy was performed with excision of the masses and complete rehabilitation of the patient.

Research Summary

Extramedullary hematopoiesis (EMH) can occur in patients with thalassemia intermedia as a physiologic response to insufficient erythropoiesis to meet circulatory demands. 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.

Practical Implications

Diagnostic Awareness

Spinal cord compression from EMH should be suspected in patients with chronic anemia and hematological disease presenting with neurological disorders.

Importance of Early Diagnosis

Early diagnosis of EMH will affect the course of management and may reduce the incidence of irreversible neurologic damage.

Role of MRI

MRI is considered the technique of choice for the diagnosis and evaluation of spinal cord compression resulting from EMH.

Study Limitations

1
The study is a case report, limiting generalizability.
2
Lack of long-term follow-up beyond one year post-surgery.
3
The rarity of the condition makes evidence-based guidelines for treatment challenging to establish.

Your Feedback

Was this summary helpful?

Back to Cardiovascular Science