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骨髓增生异常综合征合并自身免疫性溶血性贫血:霉酚酸酯治疗后难治性贫血缓解

Myelodysplastic syndrome complicated by autoimmune hemolytic anemia: remission of refractory anemia following mycophenolate mofetil.

作者信息

Lin J-T, Wang W-S, Yen C-C, Chiou T-J, Liu J-H, Hsiao L-T, Yang M-H, Chao T-C, Tai C-J, Chen P-M

机构信息

Division of Medical Oncology, Department of Medicine, Taipei Veterans General Hospital, #201, Sec. 2, Shi-Pai Road, Taiwan.

出版信息

Ann Hematol. 2002 Dec;81(12):723-6. doi: 10.1007/s00277-002-0539-3. Epub 2002 Nov 16.

DOI:10.1007/s00277-002-0539-3
PMID:12483369
Abstract

Autoimmune hemolytic anemia (AIHA) rarely occurs in myelodysplastic syndrome (MDS). A 36-year-old Asian female was diagnosed with MDS (refractory cytopenia with multilineage dysplasia, RCMD) and complicated by AIHA 7 months later. Secondary myelofibrosis developed at the same time. Steroid therapy was ineffective and cyclosporin A (CsA) was discontinued due to its neurotoxicity with the development of leukoencephalopathy. However, the patient achieved a good hematological response after the use of mycophenolate mofetil (MMF, CellCept) with a dose of 1 g/day and prednisolone (15 mg/day). Prednisolone was tapered off over the next 3 weeks. The patient did not require any blood support 4 weeks after the use of MMF and has been hematologically stable for 4 months. To our knowledge, this is the first report of using MMF in treating MDS complicated by AIHA. MMF might be considered as a salvage therapy for patients with refractory anemia complicated by AIHA.

摘要

自身免疫性溶血性贫血(AIHA)在骨髓增生异常综合征(MDS)中很少见。一名36岁的亚洲女性被诊断为MDS(多系发育异常的难治性血细胞减少症,RCMD),7个月后并发AIHA。同时发生了继发性骨髓纤维化。类固醇治疗无效,由于环孢素A(CsA)导致白质脑病而出现神经毒性,故停用。然而,患者在使用剂量为1 g/天的霉酚酸酯(MMF,骁悉)和泼尼松龙(15 mg/天)后获得了良好的血液学反应。泼尼松龙在接下来的3周内逐渐减量。使用MMF 4周后,患者不需要任何血液支持,并且血液学稳定已达4个月。据我们所知,这是关于使用MMF治疗并发AIHA的MDS的首例报告。MMF可被视为治疗并发AIHA的难治性贫血患者的挽救疗法。

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