Hypopigmentation with Hemophagocytic Lymphohistiocytosis: A Clinical Approach

摘要

: Langerhans Cell Histiocytosis(LCH) is a clinically diversedisease characterized by clonal proliferation and accumulation ofabnormal bone marrow derived CD 1a+/CD 207+ Langerhans cells.It is nowregarded as a myeloid neoplasm. It affects various organs i.e bone (mostcommon), skin, lungs, pituitary glands, liver, spleen, bone marrow etc.-There is ERK pathway activation due to BRAF V600E mutations in about60% LCH patients.