Ювенільна ксантогранульома

ЗаJeffrey M. Lipton, MD, PhD, Zucker School of Medicine at Hofstra/Northwell;
Carolyn Fein Levy, MD, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell
Переглянуто/перевірено жовт. 2024

Juvenile xanthogranuloma is a dendritic cell-related histiocytic syndrome that usually affects only the skin, causing single or multiple lesions, which can resolve spontaneously.

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Systemic involvement can occur, causing visceral organ lesions, involving the liver, lungs, and/or bone marrow.

Juvenile xanthogranuloma is rarely associated with neurofibromatosis and/or juvenile myelomonocytic leukemia

Topical corticosteroids or sirolimus can be used to treat skin lesions. Systemic lesions are treated with chemotherapy similar to what is used in Langerhans cell histiocytosis. Mitogen-activated protein kinase (MAPK) pathway mutations have been identified, so targeted therapy can also be used.