letterer-Siwe disease
MONDO:0009519Mondo
Findings
No curated finding names letterer-Siwe disease yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
A multifocal, multisystem form of Langerhans-cell histiocytosis. There is involvement of multiple organ systems including the bones, skin, liver, spleen, and lymph nodes. Patients are usually infants presenting with fever, hepatosplenomegaly, lymphadenopathy, bone and skin lesions, and pancytopenia.
Definition from the Mondo Disease Ontology (MONDO:0009519), read 2026-09-29. CC BY 4.0.
Where it sits
Other names
3 names
Resolves to: letterer-Siwe disease
- Also called
- acute and disseminated Langerhans cell histiocytosisacute disseminated Langerhans cell histiocytosismultifocal multisystem Langerhans cell histiocytosis