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两名地中海贫血患者因髓外造血导致脊髓压迫:输血治疗后完全消退

Spinal cord compression due to extramedullary haematopoiesis in two patients with thalassaemia: complete regression with blood transfusion therapy.

作者信息

Aliberti B, Patrikiou A, Terentiou A, Frangatou S, Papadimitriou A

机构信息

Neurology Department of Red Cross Hospital, Athens, Greece.

出版信息

J Neurol. 2001 Jan;248(1):18-22. doi: 10.1007/s004150170264.

Abstract

Extramedullary haematopoiesis, a common finding in thalassaemia, is rarely localized in the spinal cord and even more rarely has neurological manifestations. We present two patients suffering from thalassaemia (intermedia and beta homozygous) and paraparesis due to spinal cord compression from intrathoracic extramedullary haematopoietic masses. Diagnosis was based on magnetic resonance imaging findings, and treatment consisted of blood hypertransfusions. The majority of published cases have been successfully treated by radiation and in some cases by blood transfusions. Our two patients completely recovered, and there has been no recurrence during the 4 years since their treatment. Diagnosis and treatment of this rare condition are discussed.

摘要

髓外造血是地中海贫血的常见表现,很少局限于脊髓,更少有神经学表现。我们报告了两名患有地中海贫血(中间型和β纯合子型)的患者,因胸段髓外造血肿块压迫脊髓而出现双下肢轻瘫。诊断基于磁共振成像结果,治疗包括血液过度输血。大多数已发表的病例通过放疗成功治疗,在某些情况下通过输血治疗。我们的两名患者完全康复,自治疗以来的4年中未复发。本文讨论了这种罕见疾病的诊断和治疗。

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