ABPath Pediatric Pathology

Practice session

A 6-year-old has pancytopenia and hepatosplenomegaly. Bone marrow biopsy shows infiltration by Langerhans-type cells with grooved nuclei ('coffee bean'), eosinophils, and IHC positive for CD1a, S100, and langerin (CD207). BRAF V600E mutation detected. Which diagnosis?

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