Langerhans Cell Histiocytosis (LCH) Flashcards Preview

SBL Exam 1 WBCs, Spleen, and Thymus > Langerhans Cell Histiocytosis (LCH) > Flashcards

Flashcards in Langerhans Cell Histiocytosis (LCH) Deck (9)
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1

What is Langerhans cell histiocytosis?

Langerhans cell histiocytosis

-neoplasm of immature dendritic cells (Langerhans cells)

-"tennis racket" appearing Birbeck granules on electron microscopy

-CD1a+ and S-100+

2

What are the main types of Langerhans cell histocytosis?

-multifocal/multisystem (Letterer-Siwe)

-unifocal and multifocal unisystem (eosinophilic granuloma)

-pulmonary Langerhans cell histiocytosis

 

3

What is the common presentation of multifocal/multisystemic Langerhans cell histiocytosis (Letterer-Siwe disease)?

-<2 y/o; can affect adults

-skin rash resembling seborrheic reuption

-lesions present in multiple organ systems

4

What is the expected prognosis of multifocal/multisystemic Langerhans cell histiocytosis (Letterer-Siwe disease)

-rapily fatal if untreated

-w/ treatment, 50% 5 year survival

5

What is the common presentation of unifocal and multifocal unisystem Langerhans cell histiocytosis (eosinophilic granuloma)?

Eosinophils present in addition to Langerhan cells

 

Unifocal:

-patholgic fractures in adolescents

 

Multifocal unisystem:

-pathologic fractures in young children

-Hand-Schuller-Christian tirad (calverial bone defects, diabetes insipidus, exopthalmos)

6

What is the expected prognosis of unifocal and multifocal unisystem Langerhans cell histiocytosis (eosinophilic granuloma)?

spontaneous regression can occur

 

multifocal -> chemotherapy

unifocal -> excision/radiation

7

What is the common presentation of pulmonary Langerhans cell histiocytosis?

-typically adult smokers

-multiple nodules in lungs

8

What is the expected prognosis of pulmonary Langerhans cell histiocytosis?

-can regress with cesation of smoking

9

What B cell neoplasm is CD5+?

chronic lymphocytic leukemia (CLL)