AI Article Synopsis
- Congenital neutropenia is a primary immunodeficiency marked by low neutrophil counts and can be mild to severe; hematopoietic stem cell transplantation is a potential treatment but can lead to complications like graft-versus-host disease.
- A case of a 2-year-old girl with congenital neutropenia who developed scalp abscesses and cellulitis was reported, and after a transplant procedure, she was diagnosed with graft-versus-host disease, which ultimately led to her death.
- Graft-versus-host disease is a serious concern in stem cell transplants, highlighting the importance of early recognition and effective management to reduce risks of high morbidity and mortality.
Article Abstract
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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| http://dx.doi.org/10.29262/ram.v71i4.1405 | DOI Listing |
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