疼痛性脊髓压迫症作为中间型β地中海贫血相关的髓外造血的一种并发症。

Painful spinal cord compression as a complication of extramedullary hematopoiesis associated with beta-thalassemia intermedia.

作者信息

Goerner Martin, Gerull Sabine, Schaefer Erhard, Just Marianne, Sure Martin, Hirnle Peter

机构信息

Department of Hematology & Oncology, City Hospital Bielefeld, Germany.

出版信息

Strahlenther Onkol. 2008 Apr;184(4):224-6. doi: 10.1007/s00066-008-1794-6.

DOI:10.1007/s00066-008-1794-6

PMID:18398588

Abstract

BACKGROUND

Spinal cord compression due to extramedullary hematopoiesis (EMH) is a rare manifestation of thalassemia. General therapeutic recommendations do not exist.

CASE REPORT

We report a 23 year old male with beta-thalassemia intermedia and back pain resistant to analgetic medication. Multiple extradural mass lesions on MRI of the lumbar spine were identified. Hypertransfusion was initiated with no relief of symptoms. Low dose irradiation with 20 Gy had to be added to achieve adaequate response.

RESULT

With the combination of transfusion therapy and irradiation a rapid and long-lasting relief of symptoms was achieved. 12 month post irradiation the patient is free of pain and neurological symptoms.

CONCLUSION

Clinical awareness of this phenomenon and knowledge of the available treatment option is essential for successful outcome. In treatment refractory symptoms irradiation could successfully contribute to a rapid therapeutic response.

摘要