Abstract Purpose To describe a pediatric case of Evans syndrome complicated by concurrent iron deficiency anemia that presented diagnostic challenges and highlight the importance of comprehensive hematologic evaluation in pediatric cytopenias. Methods We report a 14-year-old female presenting with a 2-week history of petechiae, pallor, and fatigue following an upper respiratory infection. Laboratory evaluation included complete blood count, direct antiglobulin test (DAT), comprehensive metabolic panel, iron studies, peripheral blood smear examination, and bone marrow biopsy. Treatment response to intravenous immunoglobulin (IVIG) and corticosteroids was monitored over 20 months. Results Initial findings revealed severe thrombocytopenia (48 × 10³/µL), Coombs-positive autoimmune hemolytic anemia (hemoglobin 8.1 g/dL, DAT IgG 3+), and concurrent iron deficiency anemia (ferritin 3 ng/mL). Peripheral blood smear demonstrated microcytic hypochromic anemia with spherocytes, dacrocytes, and thrombocytopenia with giant platelets. Physical examination revealed splenomegaly. Bone marrow biopsy showed normocellular marrow with erythroid hyperplasia and depleted iron stores, excluding primary bone marrow pathology. IVIG therapy (1 g/kg × 2 days) failed to improve platelet counts, but oral prednisone (2 mg/kg/day) achieved complete hematologic remission by day 24 (platelets 581 × 10³/µL, hemoglobin 11.9 g/dL). Iron supplementation corrected the deficiency. At 20-month follow-up, sustained hematologic remission was maintained (hemoglobin 13.8 g/dL, platelets 174 × 10³/µL), though elevated TSH (7 µIU/mL) indicated steroid-related endocrine effects. Conclusion Evans syndrome can be masked by concurrent iron deficiency anemia in pediatric patients, emphasizing the need for comprehensive autoimmune evaluation including DAT testing in children presenting with cytopenias. Corticosteroids remain effective first-line therapy, but long-term monitoring for steroid-related complications is essential.
Ahmadi et al. (Tue,) studied this question.