Management of spinal cord compression caused by extramedullary hematopoiesis in beta-thalassemia.
Thalassemia is a chronic hemolytic anemia, endemic around the Mediterranean basin. Extramedullary hematopoiesis (EMH) is a normal compensatory reaction that can involve many organs or tissues, including the epidural space, leading to a spinal cord compression syndrome. In almost all cases, the clinical and MR patterns are those of a lower dorsal spinal cord progressive compression secondary to an epidural expanding process. The management still remains controversial, including mainly blood transfusions, decompressive surgery and radiotherapy. We present a case of EMH in a thalassemic adolescent boy presenting with a T12 level of compression, treated successfully with blood transfusion, surgical decompression and radiotherapy.
Moncef
Berhouma
B
Department of Neurosurgery B, Pierre Wertheimer Neurological and Neurosurgical Hospital of Lyon, Lyon, France. berhouma.moncef@yahoo.fr
Hafedh
Jemel
J
D000293:Adolescent; D001803:Blood Transfusion; D003131:Combined Modality Therapy; D006411:Hematopoiesis, Extramedullary; D006801:Humans; D007796:Laminectomy; D008159:Lumbar Vertebrae / Q000473:pathology / Q000601:surgery; D008297:Male; D013117:Spinal Cord Compression / Q000209:etiology* / Q000473:pathology / Q000628:therapy*; D013904:Thoracic Vertebrae / Q000473:pathology / Q000601:surgery; D017086:beta-Thalassemia / Q000503:physiopathology*