Early-onset Evans Syndrome in a 4-Month-Old Infant: A Case Report and Review of Literature
Khaled Kamaleddin Mohamed1, Faisal Othman Al-Qurashi1, Mohammad Hussain Al-Qahtani1
1Department of Pediatrics, King Fahd Hospital of the University, University of Dammam, Dammam, Saudi Arabia.
Insights
Evans syndrome (ES), a rare autoimmune disorder, involves autoimmune hemolytic anemia and immune thrombocytopenic purpura. This case report highlights early-onset ES in an infant, suggesting it can occur before six months of age.
Area of Science:
- Pediatrics
- Hematology
- Immunology
Background:
- Evans syndrome (ES) is a rare autoimmune condition.
- It is characterized by concurrent autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP).
- ES is seldom reported in infants, particularly those under six months.
Observation:
- A 4-month-old infant presented with acute pallor and jaundice.
- The infant had no prior family history of hematological or autoimmune diseases.
- Investigations confirmed anemia, thrombocytopenia, and a positive direct Coombs test with IgG autoantibodies.
Findings:
- The infant was treated with blood transfusion and high-dose steroids.
- The patient showed significant clinical improvement following treatment.
- This case suggests ES can manifest in early infancy.
Implications:
- This case supports the potential for early-onset Evans syndrome in infants younger than six months.
- Early diagnosis and management are crucial for favorable outcomes in pediatric ES.
- Further research into the incidence and presentation of ES in infants is warranted.
Abstract:
Evans syndrome (ES) is a rare autoimmune disorder characterized by autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP). We report a case of a 4-month old infant who presented with a history of acute pallor and jaundice. She had no family history of any hematological or autoimmune disorders. Her laboratory investigations revealed a positive direct Coombs test with immunoglobulin G autoantibodies, anemia and thrombocytopenia. She was managed initially by blood transfusion and started on high-dose steroid therapy with marked improvement. Very few cases of ES in infants have been reported in the literature. We concluded that this case report may support the possibility of an early-onset ES among infants <6 months of age.
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