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

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

Spinal cord compression (SCC) is an unusual sequela of extra-medullary hematopoiesis (EMH). We report a patient diagnosed with β-thalassemia intermedia at the age of 7 years presenting as a 24-year-old with symptoms suggestive of paraparesis. MR imaging revealed long masses of EMH opposite T5−T11 and L5−S2 vertebrae with cord compression at T6 vertebrae. Patient was treated with external beam radiotherapy (EBRT) to a low dose of 20 Gy in 10 fractions over 2 weeks. The patient had symptomatic relief of paraparesis by the 5th fraction and nearly regained full power in bilateral lower limbs by EBRT conclusion. Patient was begun on hydroxyurea post EBRT and was symptom free at 2-month follow up. With a follow-up of 18 months so far, he remains asymptomatic and free of recurrence. MRI correlation of pre-EBRT, post-EBRT and at first follow-up showed a significant reduction in the size of EMH, increase in diameter of spinal canal post EBRT but a persistent edema which had no clinical manifestation. Though there was a 58% drop in leukocyte count by the end of EBRT, there was no leukocytopenia. We suggest that EBRT should be treatment of choice for SCC due to EMH as it produces as rapid and durable response with minimal acute hematological side-effects.

Original languageEnglish
Pages (from-to)195-199
Number of pages5
JournalAnnals of palliative medicine
Volume6
Issue number2
DOIs
Publication statusPublished - 01-01-2017

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Spinal Cord Compression
beta-Thalassemia
Hematopoiesis
Radiotherapy
Paraparesis
Spine
Spinal Canal
Hydroxyurea
Leukopenia
Leukocyte Count
Lower Extremity
Edema
Recurrence

All Science Journal Classification (ASJC) codes

  • Advanced and Specialised Nursing
  • Anesthesiology and Pain Medicine

Cite this

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title = "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",
abstract = "Spinal cord compression (SCC) is an unusual sequela of extra-medullary hematopoiesis (EMH). We report a patient diagnosed with β-thalassemia intermedia at the age of 7 years presenting as a 24-year-old with symptoms suggestive of paraparesis. MR imaging revealed long masses of EMH opposite T5−T11 and L5−S2 vertebrae with cord compression at T6 vertebrae. Patient was treated with external beam radiotherapy (EBRT) to a low dose of 20 Gy in 10 fractions over 2 weeks. The patient had symptomatic relief of paraparesis by the 5th fraction and nearly regained full power in bilateral lower limbs by EBRT conclusion. Patient was begun on hydroxyurea post EBRT and was symptom free at 2-month follow up. With a follow-up of 18 months so far, he remains asymptomatic and free of recurrence. MRI correlation of pre-EBRT, post-EBRT and at first follow-up showed a significant reduction in the size of EMH, increase in diameter of spinal canal post EBRT but a persistent edema which had no clinical manifestation. Though there was a 58{\%} drop in leukocyte count by the end of EBRT, there was no leukocytopenia. We suggest that EBRT should be treatment of choice for SCC due to EMH as it produces as rapid and durable response with minimal acute hematological side-effects.",
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AU - Varma, Muralidhar

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