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一名患有肝肿大、巨舌症和紫癜的患者在自体干细胞移植后淀粉样变性相关症状的改善。

Improvement of amyloid-related symptoms after autologous stem cell transplantation in a patient with hepatomegaly, macroglossia and purpura.

作者信息

Patriarca F, Geromin A, Fanin R, Damiani D, Sperotto A, Baccarani M

机构信息

Division of Haematology and Department of Bone Marrow Transplantation, Udine University Hospital, Udine, Italy.

出版信息

Bone Marrow Transplant. 1999 Aug;24(4):433-5. doi: 10.1038/sj.bmt.1701925.

DOI:10.1038/sj.bmt.1701925
PMID:10467336
Abstract

AL amyloidosis was diagnosed in a 56-year-old woman with spontaneous purpura, macroglossia and hepatomegaly, a serum IgGk monoclonal gammopathy and a 25% plasma cell bone marrow infiltration. She was started on high-dose treatment consisting of four monthly cycles of VID chemotherapy, then underwent a stem cell collection after priming with cyclophosphamide + G-CSF. Myeloablative therapy was with melphalan and busulfan. Hematologic recovery was fast and uncomplicated. At follow-up 22 months from ASCT, the patient shows a complete remission of the clonal plasma cell disorder, normalization of liver size and alkaline phosphatase level and a significant improvement in the signs of vascular and soft tissue amyloid infiltration.

摘要

一名56岁女性被诊断为AL淀粉样变性,伴有自发性紫癜、巨舌和肝肿大,血清IgGk单克隆丙种球蛋白病以及25%的浆细胞骨髓浸润。她开始接受高剂量治疗,包括四个月周期的VID化疗,然后在环磷酰胺+粒细胞集落刺激因子预处理后进行干细胞采集。清髓性治疗采用美法仑和白消安。血液学恢复迅速且无并发症。自自体造血干细胞移植(ASCT)后22个月的随访显示,患者的克隆性浆细胞疾病完全缓解,肝脏大小和碱性磷酸酶水平恢复正常,血管和软组织淀粉样浸润体征有显著改善。

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Improvement of amyloid-related symptoms after autologous stem cell transplantation in a patient with hepatomegaly, macroglossia and purpura.一名患有肝肿大、巨舌症和紫癜的患者在自体干细胞移植后淀粉样变性相关症状的改善。
Bone Marrow Transplant. 1999 Aug;24(4):433-5. doi: 10.1038/sj.bmt.1701925.
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High-dose melphalan with autologous stem cell transplantation after VAD induction chemotherapy for treatment of amyloid light chain amyloidosis: a single centre prospective phase II study.VAD诱导化疗后大剂量美法仑联合自体干细胞移植治疗轻链型淀粉样变性:一项单中心前瞻性II期研究
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Haematologica. 2000 Jan;85(1):52-8.

引用本文的文献

1
[Macroglossia in a 53-year-old man with muscle complaints].
Hautarzt. 2013 Apr;64(4):295-7. doi: 10.1007/s00105-013-2546-9.
2
Primary systemic amyloidosis.原发性系统性淀粉样变性
Curr Treat Options Oncol. 2000 Apr;1(1):83-9. doi: 10.1007/s11864-000-0018-9.