Childhood Histiocytosis

Histiocytosis is a general name for a group of rare disorders characterized by increased numbers of blood cells called histiocytes in the blood and tissues. These cells usually help fight infection and destroy certain foreign substances in the body. When a child has histiocytosis, these cells begin to attack the body, targeting bodily organs including the bone marrow, liver, spleen, lungs, skin, bone and brain. Histiocytosis usually occurs in children, often during infancy, but also in adults.

There are two major types of histiocytosis:

• Langerhans cell histiocytosis (LCH) involves the dendritic cells, a type of histiocyte cell that is present in bodily tissues that are in contact with the external environment, such as the skin.
• Hemophagocytic lymphohistiocytosis (HLH) involves the macrophage cells, which are immune system cells that form in response to an infection or to damaged or dead cells.

Other related diseases include:

• juvenile xanthogranuloma
• Rosai Dorfman disease

Histiocytosis treatment at Dana-Farber/Boston Children's

Children with histiocytosis are treated through the Histiocytosis Program at Dana-Farber/Boston Children's Cancer and Blood Disorders Center. All senior medical staff members of our histiocytosis treatment program participate in clinical research activities, so our patients have access to the very best and up-to-date treatments available.