Langerhans Cell Histiocytosis
Background
What is the estimated annual incidence of Langerhans cell histiocytosis (LCH) in the U.S.?
~300 cases/yr of LCH in the U.S.
Is there a gender predilection in LCH?
Yes. Males are more commonly affected than females (3:2).
What are the diagnostic histopathologic characteristics of LCH?
Birbeck granules on electron microscopy and CD1a/S100 positivity are typical for LCH.
What is the normal function of Langerhans cells? Where are they normally found?
Langerhans cells serve as antigen presenting cells to lymphocytes and are typically found in skin, mucosa, spleen, and lymphatics.
What organs are typically involved in LCH?
Bones (children) and lungs (adults), but LCH can present in any organ (e.g., liver, skin, etc.).
What defines good risk groups vs. poor risk groups?
Good risk: unifocal lesion in bone (local therapy)
Poor risk: <2 yo with organ dysfunction
What is Hand-Schuller-Christian Dz?
Hand-Schuller-Christian Dz refers to the proliferation of hystiocytes that results in exophthalmos, skull lesions, diabetes insipidus (DI), and hemangiomas (poor prognosis).
How does LCH relate to other histiocytosis entities like eosinophilic granuloma, Letterer-Siwe Dz, Hand-Schuller-Christian Dz, and histiocytosis X?
Eosinophilic granuloma is an older term for focal LCH, while the eponyms represent multifocal Dz. –Histiocytosis X is the older term for LCH.
Workup/Staging
In what age group are widespread seborrheic rash in the scalp/groin, +LAD, and liver involvement seen with LCH?
These Sx are seen in LCH pts <2 yo.
For what age group is DI a common presentation of LCH?
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