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治疗性血浆置换在难治性重症温自身免疫性溶血性贫血中的作用:两例病例报告。

Role of therapeutic plasma exchanges in refractory severe warm autoimmune hemolytic anemia: Presentation of two case reports.

机构信息

Apheresis & Cellular Therapy Unit, Department of Hemotherapy and Hemostasis, ICMHO, IDIBAPS, Hospital Clínic, University of Barcelona, Barcelona, Spain.

Department of Hematology and Hemotherapy, Hospital Ramón y Cajal, Madrid, Spain.

出版信息

Transfusion. 2020 Nov;60(11):2753-2757. doi: 10.1111/trf.16143. Epub 2020 Oct 22.

DOI:10.1111/trf.16143
PMID:33089904
Abstract

BACKGROUND

Warm autoimmune hemolytic anemia (WAIHA) is a disorder with a usually good response to corticosteroid treatment, whereas in some cases first-line treatment's response is poor and other therapies such as intravenous immunoglobulins (IVIGs), rituximab, or splenectomy must be applied.

STUDY DESIGN AND METHODS

Herein, we describe two patients with severe WAIHA treated at our center, who obtained a response after therapeutic plasma exchanges (TPEs) combined with low doses of IVIG.

RESULTS

The first patient was an 18-year-old man with no relevant past medical history who was diagnosed with WAIHA. The patient presented a progressive clinical worsening despite treatment with prednisone, IVIG, and rituximab. After starting TPEs, signs of hemolysis rapidly improved and hemoglobin started to recover. The second patient was a 38-year-old man with a past history of immune thrombocytopenia and WAIHA. The patient presented a new flare of WAIHA, with no response after 2 weeks of treatment with corticosteroids, IVIG, and rituximab. After initiation of TPEs, the patient had an improvement in hemolysis biomarkers and recovery of hemoglobin concentration.

CONCLUSION

Combination of TPEs with rituximab and IVIG might be considered as a therapeutic option in patients with severe WAIHA without response to corticosteroid and IVIG treatment.

摘要

背景

自身免疫性溶血性贫血(WAIHA)通常对皮质类固醇治疗有良好的反应,但在某些情况下,一线治疗的反应不佳,必须采用其他疗法,如静脉注射免疫球蛋白(IVIGs)、利妥昔单抗或脾切除术。

研究设计与方法

本文描述了在我们中心治疗的两名患有严重 WAIHA 的患者,他们在接受治疗性血浆置换(TPEs)联合低剂量 IVIG 治疗后获得了缓解。

结果

第一例患者是一名 18 岁男性,无相关既往病史,被诊断为 WAIHA。尽管接受了泼尼松、IVIG 和利妥昔单抗治疗,但患者的临床症状仍逐渐恶化。开始 TPEs 后,溶血迹象迅速改善,血红蛋白开始恢复。第二例患者是一名 38 岁男性,既往有免疫性血小板减少症和 WAIHA 病史。该患者出现了 WAIHA 的新发作,在接受皮质类固醇、IVIG 和利妥昔单抗治疗 2 周后无反应。开始 TPEs 后,患者的溶血生物标志物改善,血红蛋白浓度恢复。

结论

对于对皮质类固醇和 IVIG 治疗无反应的严重 WAIHA 患者,联合 TPEs 与利妥昔单抗和 IVIG 可能是一种治疗选择。

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