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一名患有X连锁无丙种球蛋白血症和急性髓系白血病的患者成功进行了造血细胞移植。

Successful hematopoietic cell transplantation in a patient with X-linked agammaglobulinemia and acute myeloid leukemia.

作者信息

Abu-Arja Rolla F, Chernin Leah R, Abusin Ghada, Auletta Jeffery, Cabral Linda, Egler Rachel, Ochs Hans D, Torgerson Troy R, Lopez-Guisa Jesus, Hostoffer Robert W, Tcheurekdjian Haig, Cooke Kenneth R

机构信息

Pediatric Blood and Marrow Transplant Program, Nationwide Children's Hospital, Columbus, Ohio.

Allergy/Immunology Associates, Inc., Case Western Reserve University, Cleveland, Ohio.

出版信息

Pediatr Blood Cancer. 2015 Sep;62(9):1674-6. doi: 10.1002/pbc.25554. Epub 2015 Apr 20.

Abstract

X-linked agammaglobulinemia (XLA) is a primary immunodeficiency characterized by marked reduction in all classes of serum immunoglobulins and the near absence of mature CD19(+) B-cells. Although malignancy has been observed in patients with XLA, we present the first reported case of acute myeloid leukemia (AML) in a patient with XLA. We also demonstrate the complete correction of the XLA phenotype following allogeneic hematopoietic cell transplantation for treatment of the patient's leukemia.

摘要

X连锁无丙种球蛋白血症(XLA)是一种原发性免疫缺陷病,其特征为各类血清免疫球蛋白显著减少,且几乎不存在成熟的CD19(+) B细胞。虽然在XLA患者中已观察到恶性肿瘤,但我们报告了首例XLA患者发生急性髓系白血病(AML)的病例。我们还证明,在对该患者的白血病进行异基因造血细胞移植治疗后,XLA表型得到了完全纠正。

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