1例婴儿先天性中性粒细胞减少症和急性移植物抗宿主病。[病例报告]。

Cristian Jesús Huchim-Peña, Emiliano Catana-Gallegos, Nayibe Getsemaní Martín-Burgos, Jesús Esteban Ambrosio Pacheco-Sierra
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引用次数: 0

摘要

背景:先天性中性粒细胞减少症是一种以中性粒细胞数量异常为特征的原发性免疫缺陷。它分为轻度、中度和重度。造血干细胞移植是一种潜在的治疗干预手段;然而,移植物抗宿主病是主要的并发症。病例报告:我们报告一个2岁的女性患者,有先天性中性粒细胞减少症的历史。她有头皮脓肿和隔膜前蜂窝织炎,治疗满意。经过多学科的研究,一项接受同种异体造血祖细胞移植的方案被启动;随后被诊断为移植物抗宿主病,最终导致患者死亡。结论:移植物抗宿主病仍然是异体造血干细胞移植患者的一个重要并发症。鉴于相关发病率和死亡率的显著程度,其识别、管理和治疗是临床相关的方面。
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[Congenital neutropenia and acute graft-versus-host disease in an infant. A case report].

Background: Congenital neutropenia is a primary immunodeficiency characterized by quantitative anomalies in neutrophil counts. It is classified as mild, moderate, or severe. Hematopoietic stem cell transplantation stands as a potential therapeutic intervention; nevertheless, graft-versus-host disease emerges as a main complication.

Case report: We report the case of a 2-year-old female patient, with a history of congenital neutropenia. She had scalp abscesses and preseptal cellulitis, which were satisfactorily treated. After a multidisciplinary approach, a protocol was initiated to receive an allogenic hematopoietic progenitor cell transplant; graft versus host disease was subsequently diagnosed, ultimately resulting in death of the patient.

Conclusion: Graft-versus-host disease remains a significant complication in patients who undergo allogeneic hematopoietic stem cell transplants. Its recognition, management, and treatment are clinically relevant aspects given the substantial degree of associated morbidity and mortality.

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