The Teaching Point

Among adolescent patients with known, reportedly managed blood dyscrasias and new onset of neurological symptoms, the currently standard practices of bone marrow transplantation, chronic transfusions and HbF stimulating medications make EMH a rare occurrence. This case illustrates the value of recognizing variations in management due to secondary factors, such as a remote history of transfusion reaction or variability in response to therapy, as a catalyst for making relatively uncommon etiologies such as extramedullary hematopoiesis more likely.






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From the manuscript

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

Free full text article: Cord Compression due to Extramedullary Hematopoiesis in an Adolescent with Known Beta Thalassemia Major

Abstract
We describe a 16 year-old male with 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.






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