Manifestaciones respiratorias asociadas con neutropenia cíclica y error innato de la inmunidad (deficiencia de IgG3): Reporte de caso.

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Title:	Manifestaciones respiratorias asociadas con neutropenia cíclica y error innato de la inmunidad (deficiencia de IgG3): Reporte de caso.
Alternate Title:	Respiratory manifestations associated with cyclic neutropenia and innate immune deficiency (IgG3 deficiency): a case report.
Authors:	Rodríguez-Flores, Daniel Alejandro¹ yurddet@gmail.com, Ortiz-Jiménez, María Del Pilar¹
Source:	Revista Alergia de Mexico. jul-sep2025, Vol. 72 Issue 3, p209-211. 3p.
Subjects:	IMMUNODEFICIENCY, NEUTROPENIA, PULMONARY manifestations of general diseases, IMMUNOTHERAPY, ALLERGIC rhinitis, CHILD patients
Abstract (English):	Background: Inborn errors of immunity are a heterogeneous group of diseases caused by a diminished or absent response of one or more components of the immune system. IgG subclass deficiency is more common in childhood, especially in boys than in girls. IgG2 deficiency is the most common, followed by IgG3 deficiency. Case report: A male pediatric patient experienced respiratory distress of viral and bacterial origin and asthma in his early years, with frequent emergency room admissions and/or hospitalizations. At age 5, he was diagnosed with cyclic neutropenia, so the Pediatric Hematology Department decided to offer conservative treatment. At age 6, he was evaluated by an Otorhinolaryngologist, who diagnosed allergic rhinitis and performed a septumplasty and turbinectomy. At age 7, he was evaluated by an Allergist, with normal laboratory results: immunoglobulin profile, lymphocyte subpopulations, and blood count. Subcutaneous allergen-specific testing was positive. Treatment with subcutaneous immunotherapy was initiated. At age 9, the patient relapsed, with symptoms of upper respiratory tract infection and asthma. Laboratory studies revealed IgG3 deficiency (7.81 mg/dL), so treatment with intravenous immunoglobulin was initiated. Conclusion: The administration of human immunoglobulin and monoclonal antibodies is associated with a reduction in respiratory symptoms in patients with inborn errors of immunity and a better quality of life. [ABSTRACT FROM AUTHOR]
Abstract (Spanish):	Antecedentes: Los errores innatos de la inmunidad son un grupo heterogéneo de enfermedades causados por una respuesta disminuida o ausente de uno o más de los componente del sistema inmune. La deficiencia en subclases IgG es la más común en la niñez, principalmente en varones que en mujeres, sobre todo la deficiencia de IgG2, seguida de la deficiencia de IgG3. Reporte de caso: Paciente pediátrico, de género masculino, que en los primeros años vida tuvo cuadros de dificultad respiratoria de origen viral, bacteriano y asma, con ingreso a sala de urgencias y/o hospitalizaciones frecuentes. A los 5 años se le diagnosticó neutropenia cíclica, por lo que el servicio de Hematología pediátrica decidió ofrecer tratamiento conservador. A los 6 años fue valorado por Otorrinolaringología, quienes diagnosticaron rinitis alérgica, realizando septumplastia y turbinectomía. A los 7 años tuvo valoración por Alergología, con resultados de laboratorio: perfil de Inmunoglobulinas, subpoblaciones linfocitarias y biometría hemática normales. Las pruebas alergeno especificas subcutáneas resultaron positivas. Se inició tratamiento con Inmunoterapia subcutánea. A los 9 años tuvo recaída, con síntomas de infección de las vías aérea superiores y asma; los estudios de laboratorio informaron deficiencia de IgG3 (7.81 mg/dL), por lo que se inició tratamiento con Inmunoglobulina por vía intravenosa. Conclusión: La aplicación de inmunoglobulina humana y anticuerpos monoclonales se asocia con disminución de las manifestaciones respiratorias en pacientes con errores innato de la inmunidad y mejor calidad de vida. [ABSTRACT FROM AUTHOR]
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Database:	MedicLatina

Description
Abstract:	Background: Inborn errors of immunity are a heterogeneous group of diseases caused by a diminished or absent response of one or more components of the immune system. IgG subclass deficiency is more common in childhood, especially in boys than in girls. IgG2 deficiency is the most common, followed by IgG3 deficiency. Case report: A male pediatric patient experienced respiratory distress of viral and bacterial origin and asthma in his early years, with frequent emergency room admissions and/or hospitalizations. At age 5, he was diagnosed with cyclic neutropenia, so the Pediatric Hematology Department decided to offer conservative treatment. At age 6, he was evaluated by an Otorhinolaryngologist, who diagnosed allergic rhinitis and performed a septumplasty and turbinectomy. At age 7, he was evaluated by an Allergist, with normal laboratory results: immunoglobulin profile, lymphocyte subpopulations, and blood count. Subcutaneous allergen-specific testing was positive. Treatment with subcutaneous immunotherapy was initiated. At age 9, the patient relapsed, with symptoms of upper respiratory tract infection and asthma. Laboratory studies revealed IgG3 deficiency (7.81 mg/dL), so treatment with intravenous immunoglobulin was initiated. Conclusion: The administration of human immunoglobulin and monoclonal antibodies is associated with a reduction in respiratory symptoms in patients with inborn errors of immunity and a better quality of life. [ABSTRACT FROM AUTHOR]
ISSN:	00025151
DOI:	10.29262/ram.v72i3.1503