Ileri Talia, Azik Fatih, Ertem Mehmet, Uysal Zumrut, Gozdasoglu Sevgi
Department of Pediatric Hematology, Ankara University School of Medicine, Dikimevi, Ankara, Turkey.
J Pediatr Hematol Oncol. 2009 Sep;31(9):681-3. doi: 10.1097/MPH.0b013e3181a71843.
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.
髓外造血引起的脊髓压迫是中间型地中海贫血极为罕见的并发症。在生命的第一个十年中尚未有该并发症的病例报道,因为持续的红细胞生成导致患者体内出现大量异位骨髓。我们报告了一名9岁的中间型地中海贫血患者出现脊髓压迫的情况。我们还回顾了有关治疗方案的文献,因为对于这些患者的最佳治疗尚无共识。我们的患者接受放射治疗后再使用羟基脲,治疗成功。通过这种联合治疗,他在4年的随访期内未复发。对这种现象的临床认识以及早期治疗对于优化成功结果至关重要。