raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:254712

Familial sinus histiocytosis with massive lymphadenopathy

inactive · moved to ORPHA:168569H syndrome

Synonyms: Familial Rosaï-Dorfman disease · Familial SHML

Type
Disease
Group
Disorder
Status
inactive
Flag
Deprecated entity

This entity has been excluded from the Orphanet nomenclature of rare diseases and moved to H syndrome

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enFamilial sinus histiocytosis with massive lymphadenopathyFamilial Rosaï-Dorfman disease · Familial SHML
frHistiocytose sinusale familiale avec lymphadénopathie massiveHSLM familiale · Maladie de Rosaï-Dorfman familiale · SHML familiale
deSinushistiozytose, familiäre, mit massiver LymphadenopathieRosaï-Dorfman-Krankheit, familiäre · SHML, familiär
esHistiocitosis sinusal familiar con linfadenopatía masivaEnfermedad de Rosaï-Dorfman familiar · SHML familiar
itIstiocitosi sinusale familiare associata a linfadenopatia massivaMalattia di Rosaï-Dorfman familiare · SHML familiare
nlFamiliale sinus histiocytose met massieve lymfadenopathieFamiliale SHML · Familiale ziekte van Rosaï-Dorfmann
ptHistiocitose sinusal familiar com linfadenopatia maciçaDoença de Rosaï-Dorfman familiar · SHML familiar
plRodzinna histiocytoza zatokowa z masywną limfadenopatiąRodzinna choroba Rosaï i Dorfmana · Rodzinna SHML
csFamiliární sinus hystiocytosis s masivní lymfadenopatiíFamiliární Rosaïova-Dorfmanova choroba · Familiární SHML · Familiární Rosaï-Dorfmanova choroba
ukСімейний гістіоцитоз синусів з масивною лімфаденопатієюСімейна хвороба Розаї-Дорфмана · Сімейний SHML
zh家族性窦组织细胞增生伴巨大淋巴结病家族性Rosaï-Dorfman病
trMasif lenfadenopati ile seyreden ailesel sinüs histiyositozuAilesel SHML · Ailesel Rosaï-Dorfman hastalığı
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Cross-references

qualifier = Orphanet mapping relation

No cross-references in this release.

3

Classification

Not placed in any Orphanet classification in this release.

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

0 records

No prevalence record in this release.

6

HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

7

Sources for this card