Extramedullary hematopoiesis (EMH) is a normal compensatory reaction that occurs in almost all chronic hemolytic anemia, especially in transfusion independent thalassemia intermedia, which can involve many organs or tissues, including the epidural space leading to spinal cord compression syndrome. We present a case of EMH in a 29 year old woman with thalassemia major, regularly transfused since the time of diagnosis (21 months), who presented a sudden muscle weakness, difficulty walking and maintaining the upright position. Magnetic Resonance Imaging (MRI) of the thoracic spine showed spinal cord compression secondary to extramedullary hematopoiesis in the spinal canal, leading to an early diagnosis. The neurosurgical treatment (decompressive laminectomy D3-D6) in our patient led to a significant and rapid recovery. The next two MRI of the spine (after 6 and 18 months) were both negative for recurrence.
A Regularly-Transfused Patient with Thalassemia Major
GIOVANNINI, Michela
2011
Abstract
Extramedullary hematopoiesis (EMH) is a normal compensatory reaction that occurs in almost all chronic hemolytic anemia, especially in transfusion independent thalassemia intermedia, which can involve many organs or tissues, including the epidural space leading to spinal cord compression syndrome. We present a case of EMH in a 29 year old woman with thalassemia major, regularly transfused since the time of diagnosis (21 months), who presented a sudden muscle weakness, difficulty walking and maintaining the upright position. Magnetic Resonance Imaging (MRI) of the thoracic spine showed spinal cord compression secondary to extramedullary hematopoiesis in the spinal canal, leading to an early diagnosis. The neurosurgical treatment (decompressive laminectomy D3-D6) in our patient led to a significant and rapid recovery. The next two MRI of the spine (after 6 and 18 months) were both negative for recurrence.I documenti in SFERA sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
