A Case of Rare Letterer-Siwe Disease and a Literature Review

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Abstract

This case report describes a 1-month-old and 23-day-old male infant who was admitted with anorexia, cough, and respiratory distress. Upon admission, CT revealed exacerbated bilateral pulmonary inflammation, the formation of pulmonary bullae, and multi-organ involvement, including the liver and pancreas. A skin biopsy supported the diagnosis of Langerhans Cell Histiocytosis (LCH). Further evaluation confirmed Letterer-Siwe disease, categorizing the patient into the high-risk group, and initial chemotherapy was initiated according to the SCMC-LCH-2018 protocol. After three months of inpatient treatment, the infant was discharged against medical advice and subsequent follow-up confirmed mortality. This report discusses the clinical manifestations, diagnosis, and treatment of Letterer-Siwe disease in the context of this case.

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