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一名骨髓增生异常综合征患者的自身免疫性溶血性贫血:对5-氮杂胞苷治疗有反应。

Autoimmune hemolytic anemia in a patient with myelodysplastic syndrome: Responding to 5-azacitidine therapy.

作者信息

Kaur Jaspreet, Tuler Shahaf, Dasanu Constantin A

机构信息

Department of Medicine, Eisenhower Health, Rancho Mirage, CA, USA.

Lucy Curci Cancer Center, Eisenhower Health, Rancho Mirage, CA, USA.

出版信息

J Oncol Pharm Pract. 2021 Oct;27(7):1775-1778. doi: 10.1177/1078155220987616. Epub 2021 Jan 14.

Abstract

INTRODUCTION

Autoimmune disorders, including autoimmune cytopenias, are more common in patients with myelodysplastic syndrome (MDS) and may share with MDS the same steps of pathogenesis. Some patients with MDS have antibodies against red cells.

CASE REPORT

We describe herein a 79-year-old patient who presented with fatigue, jaundice and pancytopenia. She was diagnosed with warm-antibody autoimmune hemolytic anemia (AIHA) and synchronous MDS.Management and outcome: In our patient, AIHA responded to the hypomethylating agent 5-azacitidine used for the treatment of MDS. Six months later, the patient remains in clinico-laboratory remission for both MDS and AIHA.

DISCUSSION/CONCLUSIONS: Our case indirectly suggests that 5-azacitidine led to a decrease in autoantibody production by the auto-reactive B-cell clone in MDS leading in turn to a diminished rate of autoimmune hemolysis. If our observation is accurate, we believe that similar reports will populate the scientific literature in the future years.

摘要

引言

自身免疫性疾病,包括自身免疫性血细胞减少症,在骨髓增生异常综合征(MDS)患者中更为常见,并且可能与MDS具有相同的发病机制步骤。一些MDS患者存在抗红细胞抗体。

病例报告

我们在此描述一名79岁的患者,她出现疲劳、黄疸和全血细胞减少。她被诊断为温抗体自身免疫性溶血性贫血(AIHA)并同时患有MDS。

治疗与结果

在我们的患者中,AIHA对用于治疗MDS的低甲基化药物5-氮杂胞苷有反应。六个月后,患者的MDS和AIHA均处于临床实验室缓解状态。

讨论/结论:我们的病例间接表明,5-氮杂胞苷导致MDS中自身反应性B细胞克隆产生的自身抗体减少,进而导致自身免疫性溶血率降低。如果我们的观察准确,我们相信未来几年类似的报告将会充斥科学文献。

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