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[Asthma and cyclic neutropenia]
A N Salazar Cabrera1, R Berrón Pérez, J A Ortega Martell
1Departamento de Inmunología Clínica, Instituto Nacional de Pediatría, México.
Abstract:
We report a male with history of recurrent infections (recurrent oral aphtous disease [ROAD], middle ear infections and pharyngo amigdalitis) every 3 weeks since he was 7 months old. At the age of 3 years cyclic neutropenia was diagnosed with cyclic fall in the total neutrophil count in blood smear every 21 days and prophylactic antimicrobial therapy was indicated. Episodic events every 3 weeks of acute asthma and allergic rhinitis were detected at the age of 6 years old and specific immunotherapy to Bermuda grass was given during 3 years with markedly improvement in his allergic condition but not in the ROAD. He came back until the age of 16 with episodic acute asthma and ROAD. The total neutrophil count failed to 0 every 21 days and surprisingly the total eosinophil count increased up to 2,000 at the same time, with elevation of serum IgE (412 Ul/mL). Specific immunotherapy to D.pt. and Aller.a. and therapy with timomodulin was indicated. After 3 months we observed clinical improvement in the asthmatic condition and the ROAD disappeared, but the total neutrophil count did not improve. We present this case as a rare association between 2 diseases with probably no etiological relationship but may be physiopatological that could help to understand more the pathogenesis of asthma.
Insights
This case study details a patient with cyclic neutropenia experiencing recurrent infections, asthma, and oral aphtous disease. Treatment improved asthma and oral aphtous disease but not neutrophil counts, suggesting a complex interplay.
Area of Science:
- Immunology
- Hematology
- Allergy and Clinical Immunology
Background:
- Cyclic neutropenia is a rare disorder characterized by periodic drops in neutrophil counts, increasing susceptibility to infections.
- Recurrent oral aphtous disease (ROAD), asthma, and allergic rhinitis are common conditions that can significantly impact quality of life.
Observation:
- A male patient presented with a history of recurrent infections since infancy, diagnosed with cyclic neutropenia at age 3.
- At age 6, he developed episodic asthma and allergic rhinitis, followed by recurrent oral aphtous disease (ROAD) and asthma exacerbations at age 16.
- During exacerbations, neutrophil counts dropped cyclically, while eosinophil counts and IgE levels increased significantly.
Findings:
- Specific immunotherapy and timomodulin therapy led to clinical improvement in asthma and resolution of ROAD.
- Despite clinical improvements, cyclic neutropenia persisted, indicating a complex relationship between the conditions.
- The case highlights a potential physiopathological link between cyclic neutropenia, allergic diseases, and recurrent oral aphtous disease.
Implications:
- This case may offer insights into the pathogenesis of asthma and recurrent oral aphtous disease (ROAD) in the context of immune dysregulation.
- Understanding such rare associations can guide future research into the complex interplay of hematological and immunological disorders.
- Further investigation is warranted to elucidate the underlying mechanisms connecting cyclic neutropenia with allergic and autoimmune conditions.