letterer-Siwe disease

Summary

letterer-Siwe disease (MONDO:0009519) is a disease. A subtype of Langerhans cell histiocytosis specific to childhood — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	letterer-Siwe disease
Mondo ID	MONDO:0009519
OMIM	246400
Orphanet	99870
ICD-10-CM	C96.0
ICD-11	1827474596
NCIT	C3160
UMLS	C0023381
MedGen	7311
GARD	0024676
MedDRA	10024265
Is cancer (heuristic)	no

Also known as: acute and disseminated Langerhans cell histiocytosis · acute disseminated Langerhans cell histiocytosis · letterer-Siwe disease · multifocal multisystem Langerhans cell histiocytosis

Data availability: 1 cell line.

Disease family

This is a subtype of Langerhans cell histiocytosis specific to childhood. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › immune system disorder › lymphoid system disorder › lymphatic system disorder › histiocytosis › Langerhans cell histiocytosis › Langerhans cell histiocytosis specific to childhood › letterer-Siwe disease

Related subtypes (1): Hashimoto-Pritzker syndrome

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 0.