Langerhans Cell Histiocytosis Flashcards
A 1 year old infant presents to the peds clinic with the main complaint of skin rash. X ray shows cystic skeletal defects. What does he have? Is this benign or fatal? Which age group does this present in?
Letterer-Siwe disease. Malignant. Multiple organs involved. Rapidly fatal.
Where are Langerhans cells derived from? What do they do? How does Langerhans cell histiocytosis look under electron microscope? What are the cells positive for in immunohistochemistry?
Derived from bone marrow monocytes. Present antigen to naive T cells. Characteristic Birbeck granules. Cells are CD1+ and S100 by IHC.
A 4 year old infant presents to the peds clinic with the main complaint of scalp rash. X ray shows lytic skull defects. He also has diabetes Insipidus and exophthalmos. What does he have? Is this benign or fatal? Which age group does this present in?
Hand-Schüller-Christian disease. Malignant proliferation of Langerhans cells. >3 yo.
A 15 year old adolescent presents to the peds clinic with fractures. He does not have skin rash. X ray shows cystic skeletal defects. What does he have? Is this benign or fatal? Which age group does this present in? What does biopsy show?
Eosinophilic granuloma. Benign proliferation of Langerhans cells. Pathologic fracture in adolescent. Biopsy shows Langerhans cells with mixed inflammatory cells, including numerous eosinophils.
