Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) is a systemic disease associated with the proliferation and accumulation (usually in granulomas) of Langerhans cells in various tissues.

Immunodeficiency

Failure of the immune system to protect the body adequately from infection, due to the absence or insufficiency of some component process or substance.


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PMID (PMCID)
8214798
 FEMALE Infant
A 13-month-old child with chronic diarrhea, weight loss, and tachypnea.
Knutsen AP, Goodman GM.
Ann Allergy. 1993;71(4):352-6.
It is advisable that patients newly diagnosed with Langerhans cell histiocytosis be evaluated to screen for immunodeficiency.
1356892
 OTHER Adult
A case of Langerhans histiocytosis with HIV-like immunodeficiency.
Manna A, Porcellini A, Marelli A, Bianchini E, Pialoux G.
Haematologica. 1992;77(1):73-5.
To our knowledge the immunodeficiency detected in histiocytosis X affects the T suppressor lymphocyte subset, so we thought this peculiar case was worth describing.
3307293
 MIXED_SAMPLE Infant
Immunohistochemical and ultrastructural studies on histiocytosis in children.
Wakuya J.
Acta Pathol Jpn. 1987;37(6):901-13.
Thirty-four cases of eosinophilic granulomas, 18 cases of diffuse histiocytosis-X, 2 cases of Letterer-Siwe-like syndrome with immunodeficiency, 4 cases of malignant histiocytosis and virus associated hemophagocytic syndrome were studied.