Diagnosis: Langerhans cell histiocytosis
LCH is a neoplastic proliferation of LCs with highly variable clinical presentation from localized involvement of a single bone to a widely disseminated life-threatening disease.
In multisystem disease, the skin, bone, liver, spleen and bone marrow (BM) are the preferential sites of involvement.
Morphology: LCH cells with grooved or folded nuclei, inflammatory background of eosinophils, neutrophils, histiocytes and small lymphocytes.
IHC: positive for CD1a, s100, Langerin
Clinical course and prognosis: unifocal-, usually in young children or adults, high survival; multisystem disease- usually in infants, high mortality
LCH is a neoplastic proliferation of LCs with highly variable clinical presentation from localized involvement of a single bone to a widely disseminated life-threatening disease.
In multisystem disease, the skin, bone, liver, spleen and bone marrow (BM) are the preferential sites of involvement.
Morphology: LCH cells with grooved or folded nuclei, inflammatory background of eosinophils, neutrophils, histiocytes and small lymphocytes.
IHC: positive for CD1a, s100, Langerin
Clinical course and prognosis: unifocal-, usually in young children or adults, high survival; multisystem disease- usually in infants, high mortality