Langerhans Cell Histiocytosis Flashcards
What are Langerhans cells?
Specialized dendritic cells found predominantly in the skin
Where are Langerhans cells derived from?
Bone marrow monocytes
What is the role of Langerhans cells?
Present antigen to naïve T cells
What is the characteristic finding seen on electron microscopy seen with Langerhans cell histiocytosis (a neoplastic proliferation of Langerhans cells)?
Birbeck granules (tennis racket)
What are the cells of Langerhans cell histiocytosis (a neoplastic proliferation of Langerhans cells) positive for, on immunohistochemistry?
CD1a and S100
A disease involving malignant proliferation of Langerhans cells that classically present as a skin rash and cystic skeletal defects in an infant (
Letterer-Siwe Disease
A benign proliferation of Langerhans cells in bone that classically presents as pathologic fracture in adolescents?
Eosinophilic Granuloma
What is seen on biopsy of patients with eosinophilic granuloma?
Langerhans cells with mixed inflammatory cells—numerous eosinophils (Hints the name)
A malignant proliferation of Langerhans cells with the classic presentation of a scalp rash, lytic skull defects, diabetes insipidus and exophthalmos in a child (>3 y/o)?
Hand-Schuller-Christian disease
What are the 3 types of Langerhans cell histiocytosis, a neoplastic proliferation of Langerhans cells?
Letterer-Siwe disease
Eosinophilic granuloma
Hand-Schuller-Christian disease
Which of the Langerhans cell histiocytosises, a neoplastic proliferation of Langerhans cells, are malignant?
Letterer-Siwe Disease
Hand-Schuller-Christain-disease
They have someone’s name in them so they must be important and malignant
Which of the Langerhans cell histiocytosises, a neoplastic proliferation of Langerhans cells, are benign?
Eosinophilic granuloma
No ones name within this disease so it must be benign
