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异基因造血干细胞移植后吉兰-巴雷综合征的治疗性血浆置换作用:两例报告。

Role of Therapeutic Plasma Exchange in Guillain-Barre Syndrome after Allogeneic Hematopoietic Stem Cell Transplant: Report of Two Cases.

出版信息

Clin Lab. 2021 Mar 1;67(3). doi: 10.7754/Clin.Lab.2020.200613.

Abstract

BACKGROUND

Guillain-Barre Syndrome (GBS) is an acute inflammatory polyneuropathy characterized with rapid, progressive, ascending, and symmetrical weakness and areflexia. It is supposed to be an autoimmune disease related with production of antibodies by T lymphocytes activated against antigenic proteins of the peripheral nerves. Guillain-Barre Syndrome occurring after hematopoietic stem cell transplant (HSCT) has been associated with viral infections or toxic effects of chemotherapy.

METHODS

We report two GBS cases after HSCT treated successfully by means of therapeutic plasma exchange.

RESULTS

In a total of 257 patients, 2 cases (0.8%) were diagnosed with GBS following HSCT. Allogeneic HSCT was performed and complete remission was achieved. Diagnosis of GBS was established on the 45th and 69th days with respect to clinical, cerebrospinal fluid, and electromyography findings. Patients did not respond to treatment consisting of intravenous immunoglobulins (IVIG) (1 g/kg/day) for 2 days and methylprednisolone (1 g/kg/day). Mechanical ventilation was indicated in one patient due to the involvement of respiratory muscles. Therapeutic plasma exchange resulted in complete recovery in both cases.

CONCLUSIONS

Guillain-Barre Syndrome is a rare but serious complication, which may occur after HSCT. Increased awareness and early diagnosis are crucial in the management of GBS. First line treatment consists of IVIG and steroids and therapeutic plasma exchange must be considered without delay in refractory cases.

摘要

背景

格林-巴利综合征(GBS)是一种急性炎症性多发性神经病,其特征为迅速、进行性、上升性和对称性无力伴反射消失。它被认为是一种自身免疫性疾病,与 T 淋巴细胞激活对抗周围神经抗原性蛋白产生抗体有关。造血干细胞移植(HSCT)后发生的格林-巴利综合征与病毒感染或化疗的毒性作用有关。

方法

我们报告了两例成功接受治疗性血浆置换治疗的 HSCT 后 GBS 病例。

结果

在总共 257 例患者中,有 2 例(0.8%)在 HSCT 后被诊断为 GBS。进行了异基因 HSCT,完全缓解。根据临床、脑脊液和肌电图检查结果,分别在第 45 天和第 69 天确诊为 GBS。患者对静脉注射免疫球蛋白(IVIG)(1 g/kg/天)和甲泼尼龙(1 g/kg/天)治疗 2 天无反应。由于呼吸肌受累,一名患者需要机械通气。两种情况下均采用治疗性血浆置换,均完全恢复。

结论

格林-巴利综合征是一种罕见但严重的并发症,可能发生在 HSCT 后。提高认识和早期诊断对 GBS 的治疗至关重要。一线治疗包括 IVIG 和类固醇,在难治性病例中必须毫不犹豫地考虑治疗性血浆置换。

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