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[Evans syndrome in infants].
Olivia Alejandra Flores-Montes1, Martha Cecilia Escobar-Orduño1, Mónica Lozano-Garcidueñas2
1Departamento de Pediatría, Hospital General Regional Número Uno, Instituto Mexicano del Seguro Social, Ciudad Obregón, Sonora, México.
Boletin Medico Del Hospital Infantil De Mexico
|February 1, 2018
Summary
Evans syndrome, a rare autoimmune disorder, involves multiple blood cell reductions. Early diagnosis and treatment with steroids and immunoglobulin are crucial for managing this condition in infants.
Area of Science:
- Pediatric Hematology
- Autoimmune Disorders
- Clinical Case Studies
Background:
- Evans syndrome is defined by the reduction of at least two blood cell lineages without other identifiable causes.
- It involves the simultaneous or sequential occurrence of autoimmune hemolytic anemia and immune thrombocytopenia.
- The condition has a reported incidence of 37% and a mortality rate of 10%.
Observation:
- This report details the clinical course of Evans syndrome in two infants initially diagnosed with immune thrombocytopenia.
- Complementary studies confirmed the presence of hematological disorders in both cases.
- Both infants received treatment with corticosteroids and intravenous immunoglobulin.
Findings:
- Pediatricians managing thrombocytopenia must investigate other blood cell lineage disorders.
- The reported cases presented with autoimmune hemolytic anemia and monocytosis.
- Infectious and immunological studies are essential for comprehensive diagnosis.
Implications:
- Corticosteroids are the recommended first-line treatment for Evans syndrome.
- Intravenous immunoglobulin is a viable option for severe immune thrombocytopenia associated with the syndrome.
- Prompt and thorough evaluation is critical for effective management and improved outcomes in pediatric patients.
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