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一例 7 号单体性骨髓增生异常综合征伴 B 细胞减少的女孩成功进行了脐带血移植。

Successful cord blood transplantation in a girl with monosomy 7 myelodysplastic syndrome and reduced numbers of B cells.

机构信息

Department of Pediatrics, Chang Gung Children's Hospital, Chang Gung University, Taoyuan, Taiwan.

出版信息

Int J Hematol. 2010 May;91(4):705-7. doi: 10.1007/s12185-010-0548-2. Epub 2010 Mar 17.

DOI:10.1007/s12185-010-0548-2
PMID:20232178
Abstract

This report described unrelated umbilical cord blood transplantation for a 3-year-old girl with myelodysplastic syndrome and monosomy 7. The patient had a prolonged course characterized by recurrent infection and slowly progressive pancytopenia. She had reduced numbers of circulating B cells but no decline in immunoglobulin levels. Chemotherapy was not initially recommended because it was contraindicated due to intercurrent lower respiratory tract infection. After 10 months, the girl achieved hematologic remission after induction chemotherapy. The patient then underwent 2-loci HLA-mismatched unrelated donor cord blood transplantation. The time to neutrophil and platelet engraftment was 12 and 23 days post-transplantation, respectively. Acute graft-versus-host disease following transplantation was minimal. She was in continuing hematological remission with full donor chimerism 3 years after transplantation.

摘要

本报告描述了一例 3 岁女性患有骨髓增生异常综合征伴 7 号单体的非相关脐带血移植。该患者病程迁延,表现为反复感染和进行性全血细胞减少。她外周血循环 B 细胞数量减少,但免疫球蛋白水平没有下降。最初不建议化疗,因为并发下呼吸道感染而禁忌。10 个月后,患者经诱导化疗后达到血液学缓解。然后患者接受了 2 个 HLA 错配的非亲缘脐带血移植。移植后中性粒细胞和血小板植入的时间分别为 12 天和 23 天。移植后急性移植物抗宿主病较轻。移植后 3 年,患者持续处于血液学缓解状态,完全供者嵌合。

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本文引用的文献

1
Unrelated donor umbilical cord blood transplantation in pediatric myelodysplastic syndrome: a single-center experience.无关供者脐血移植治疗儿童骨髓增生异常综合征:单中心经验
Biol Blood Marrow Transplant. 2009 Aug;15(8):948-55. doi: 10.1016/j.bbmt.2009.04.010.
2
The evolution of hematopoietic SCT in myelodysplastic syndrome.骨髓增生异常综合征中造血干细胞移植的演变
Bone Marrow Transplant. 2009 Apr;43(8):597-609. doi: 10.1038/bmt.2009.28. Epub 2009 Mar 2.
3
Allogeneic stem cell transplantation for adults with myelodysplastic syndromes: importance of pretransplant disease burden.
异基因干细胞移植治疗成人骨髓增生异常综合征:移植前疾病负担的重要性。
Biol Blood Marrow Transplant. 2009 Jan;15(1):30-8. doi: 10.1016/j.bbmt.2008.10.012.
4
Treatment options in advanced myelodysplastic syndrome, with emphasis on epigenetic therapy.晚期骨髓增生异常综合征的治疗选择,重点是表观遗传治疗。
Int J Hematol. 2007 Nov;86(4):306-14. doi: 10.1532/IJH97.07034.
5
Myelodysplastic syndromes in children: a critical review of issues in the diagnosis and classification of 887 cases from 13 published series.儿童骨髓增生异常综合征:对13个已发表系列中887例病例诊断和分类问题的批判性综述
Arch Pathol Lab Med. 2007 Jul;131(7):1110-6. doi: 10.5858/2007-131-1110-MSICAC.
6
Myelodysplastic syndrome associated with monosomy 7 in childhood: a retrospective study.儿童伴7号染色体单体的骨髓增生异常综合征:一项回顾性研究。
Cancer Genet Cytogenet. 2006 Nov;171(1):72-5. doi: 10.1016/j.cancergencyto.2006.06.010.
7
Poor clinical course in a child with myelodysplastic syndrome and del(13)(q14q22).一名患有骨髓增生异常综合征和13号染色体长臂缺失(q14q22)的儿童临床病程不佳。
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8
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Haematologica. 2005 Oct;90(10):1339-45.
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Refractory anemia in childhood: a retrospective analysis of 67 patients with particular reference to monosomy 7.儿童难治性贫血:67例患者的回顾性分析,特别提及7号染色体单体。
Blood. 2003 Sep 15;102(6):1997-2003. doi: 10.1182/blood-2002-11-3444. Epub 2003 May 22.