Abstract
RATIONALE: Systemic lupus erythematosus (SLE) is a rare but serious autoimmune disorder that can present with diverse clinical manifestations, often making diagnosis challenging, particularly in pediatric patients. While thrombocytopenia is a known hematological manifestation of SLE, its occurrence as the sole initial symptom is uncommon. PATIENT CONCERNS: An 11-year-old male presented with a 2-week history of painless localized bleeding, ecchymosis, and petechiae. He also experienced mild knee joint pain but had no fever, weight loss, or other systemic symptoms. Despite initial treatment for immune thrombocytopenia (ITP), his platelet count remained persistently low, raising concern for an underlying autoimmune etiology. DIAGNOSIS: After a thorough workup, including negative blood cultures and a bone marrow biopsy that revealed megakaryocytic thrombocytopenia, autoimmune testing revealed elevated antinuclear antibodies, antidouble-stranded DNA, and decreased complement C4 levels, leading to a diagnosis of childhood-onset SLE with isolated hematologic involvement. INTERVENTION: The patient was initially treated with intravenous immunoglobulin for the management of ITP. Following the diagnosis of SLE, he was started on hydroxychloroquine, followed by azathioprine for immunosuppression. Inflammatory control was further achieved with prednisone therapy due to persistent hematologic symptoms. OUTCOME: The patient's platelet count improved to 150,000/μL after 6 months of treatment. And the other laboratory was unremarkable, indicating effective management of SLE. The patient remained free of other organ involvement during follow-up. LESSONS: This case emphasizes the importance of considering SLE in the differential diagnosis of pediatric patients with isolated ITP, especially when the clinical picture is atypical. Early autoimmune screening, including testing for antinuclear antibodies and double-stranded DNA, can facilitate early diagnosis and timely intervention.