Langerhans cell histiocytosis is a group of conditions featuring Langerhans cell proliferation. While more often described in children, they may occur in adults. Some of the conditions are progressive and have a poor prognosis, while others have a relatively good prognosis. Certain factors can help identify patients with high risk disease.
Conditions included under the heading of Langerhans cell histiocytosis include:
(1) histiocytosis X
(2) eosinophilic granuloma
(3) Letterer-Siwe disease
(4) Hand-Schuller-Christian syndrome
(5) Hashimoto-Pritzker syndrome
(6) self-healing histiocytosis
(7) pure cutaneous histiocytosis
(8) Langerhans cell granulomatosis
(9) Type II histiocytosis
(10) nonlipid reticuloendotheliosis
Risk Factor for Progressive Disease
Relative Risk
95% Confidence Interval
osseous plus mucocutaneous involvement
40.7
3.1 – 534
involvement of both osseous and extraosseous sites
37.3
21.2 – 65.5
relapse as multisystemic disease after treatment for osseous disease
37.2
4.8 – 288.4
bone involvement of >= 3 bone sites
6.1
2.2 – 16.9
mucous membrane disease
5.1
2.4 – 11.0
hepatosplenomegaly in a patient < 3 years of age at presentation
4.5
0.8 – 26.4
pituitary-thalamic involvement in the presence of multisystemic disease
2.2
0.9 – 5.3
< 5 years of age at presentation
2.1
0.9 – 4.8
>= 3 body systems involved
1.8
0.5 – 4.7
from Table 7, page 2287.
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