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
Pediatric Blood and Marrow Transplant Program, Nationwide Children's Hospital, Columbus, Ohio.
Allergy/Immunology Associates, Inc., Case Western Reserve University, Cleveland, Ohio.
Pediatr Blood Cancer. 2015 Sep;62(9):1674-6. doi: 10.1002/pbc.25554. Epub 2015 Apr 20.
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.
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。