Cord Compression due to Extramedullary Hematopoiesis in an Adolescent with Known Beta Thalassemia Major

Authors

  • Salil Soman
  • David L Rosenfeld
  • SUDIPTA ROYCHOWDHURY
  • Richard A DRACHTMAN
  • Alan COHLER

DOI:

https://doi.org/10.3941/jrcr.v3i1.83

Keywords:

Adolescent extramedullary hematopiesis, cord compression, thalassemia

Abstract

We describe a 16 year-old male with íŸ thalassemia major and gait disturbances that had not been given blood transfusions due to a severe childhood transfusion reaction. Thoracic spine MRI demonstrated hematopoietic marrow throughout the spine and epidural masses causing cord compression consistent with extramedullary hematopoiesis (EMH). After treatment with steroids, radiotherapy and monitored blood transfusions, the patient demonstrated significant improvement of his paraspinal lesions and near complete resolution of his neurological symptoms. While EMH causing cord compression in adolescents is rare in the current era of bone marrow transplantation or chronic transfusions, it should be considered when thalassemia major patients present with neurological deficits. The well defined imaging features of EMH can play a central role in its diagnosis and management, especially because surgical and / or radiotherapeutic intervention are often considered in cases of failed medical treatment.

Author Biographies

Salil Soman

UMDNJ RWJMS

Radiology Resident PGY-4

David L Rosenfeld

CLINICAL ASSISTANT PROFESSOR OF RADIOLOGY
UMDNJ – ROBERT WOOD JOHNSON MEDICAL SCHOOL

SUDIPTA ROYCHOWDHURY

CLINICAL ASSISTANT PROFESSOR OF RADIOLOGY
UMDNJ – ROBERT WOOD JOHNSON MEDICAL SCHOOL

Richard A DRACHTMAN

 

Chief - Division of Pediatric Hematology & Oncology

THE CANCER INSTITUTE OF NEW JERSEY

 

Alan COHLER

 

DEPARTMENT OF RADIATION ONCOLOGY

THE CANCER INSTITUTE OF NEW JERSEY

 

Published

2009-01-04

Issue

Section

Pediatric Radiology