title: Extramedullary hematopoiesis with spinal cord compression in a child with thalassemia intermedia.
abstract: Spinal cord compression due to extramedullary hematopoiesis is an extremely rare complication of thalassemia intermedia. No cases with this complication have been reported in the first decade of life, because masses of heterotropic marrow developed in patients as a result of continuous erythropoiesis. We report the 9-year-old patient suffering from thalassemia intermedia and presenting spinal cord compression. We also review the literature about treatment options, because there is no consensus about the optimal treatment of these patients. Our patient was successfully treated with radiation therapy followed by hydroxyurea. With this combination therapy, he had no recurrence during the 4-year follow-up period. Clinical awareness of this phenomenon with the early treatment is essential for optimizing the successful outcome.
authors.affiliation: Department of Pediatric Hematology, Ankara University School of Medicine, Dikimevi, Ankara, Turkey. taliaileri@yahoo.com
authors.forename: Talia
authors.initials: T
authors.lastname: Ileri
authors.forename: Fatih
authors.initials: F
authors.lastname: Azik
authors.forename: Mehmet
authors.initials: M
authors.lastname: Ertem
authors.forename: Zumrut
authors.initials: Z
authors.lastname: Uysal
authors.forename: Sevgi
authors.initials: S
authors.lastname: Gozdasoglu
mesh_terms: D001789:Blood Group Antigens / Q000276:immunology; D002648:Child; D003131:Combined Modality Therapy; D000075202:Contraindications; D003603:Cytotoxins / Q000627:therapeutic use; D019299:Decompression, Surgical; D004920:Erythropoiesis / Q000187:drug effects; D006411:Hematopoiesis, Extramedullary / Q000187:drug effects / Q000528:radiation effects; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D008159:Lumbar Vertebrae; D008279:Magnetic Resonance Imaging; D008297:Male; D011183:Postoperative Complications; D012447:Sacrum; D013117:Spinal Cord Compression / Q000209:etiology* / Q000532:radiotherapy; D013156:Splenectomy; D065227:Transfusion Reaction; D017086:beta-Thalassemia / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology / Q000628:therapy
chemical_list: D001789:Blood Group Antigens; D003603:Cytotoxins; D006918:Hydroxyurea