Langerhans Cell Histiocytosis Flashcards Preview

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Flashcards in Langerhans Cell Histiocytosis Deck (12):
1

What are Langerhans cells?

Specialized dendritic cells found predominantly in the skin

2

Where are Langerhans cells derived from?

Bone marrow monocytes

3

What is the role of Langerhans cells?

Present antigen to naïve T cells

4

What is the characteristic finding seen on electron microscopy seen with Langerhans cell histiocytosis (a neoplastic proliferation of Langerhans cells)?

Birbeck granules (tennis racket)

5

What are the cells of Langerhans cell histiocytosis (a neoplastic proliferation of Langerhans cells) positive for, on immunohistochemistry?

CD1a and S100

6

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

7

A benign proliferation of Langerhans cells in bone that classically presents as pathologic fracture in adolescents?

Eosinophilic Granuloma

8

What is seen on biopsy of patients with eosinophilic granuloma?

Langerhans cells with mixed inflammatory cells—numerous eosinophils (Hints the name)

9

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

10

What are the 3 types of Langerhans cell histiocytosis, a neoplastic proliferation of Langerhans cells?

Letterer-Siwe disease
Eosinophilic granuloma
Hand-Schuller-Christian disease

11

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

12

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