Histiocytosis-Lymphadenopathy Plus Syndrome (HLAS)

Alias:

H Syndrome

Shml

Histiocytosis with Joint Contractures and Sensorineural Deafness

Faisalabad Histiocytosis

Rosai-Dorfman Disease

Pigmented Hypertrichosis with Insulin-Dependent Diabetes Mellitus

Sinus Histiocytosis and Massive Lymphadenopathy

Phid

Hjcd

Sinus Histiocytosis with Massive Lymphadenopathy

Familial Rosai-Dorfman Disease

Hyperpigmentation, Cutaneous, with Hypertrichosis, Hepatosplenomegaly, Heart Anomalies, and Hypogonadism with or Without Hearing Loss

Histiocytosis and Lymphadenopathy with or Without Cutaneous, Cardiac, and/or Endocrine Features, Joint Contractures, and/or Deafness

Cutaneous Hyperpigmentation with Hypertrichosis, Hepatosplenomegaly, Heart Anomalies, and Hypogonadism with or Without Hearing Loss

Histiocytosis and Lymphadenopathy with or Without Cutaneous, Cardiac, and/or Endocrine Features, Joint Contractures and/or Deafness

Cutaneous Hyperpigmentation with Hypertrichosis Hepatosplenomegaly Heart Anomalies and Hypogonadism with or Without Hearing Loss

Histiocytosis and Lymphadenopathy with or Without Cutaneous Cardiac and/or Endocrine Features Joint Contractures and/or Deafness

Rosai-Dorfman Disease, Familial

Destombes-Rosaï-Dorfman Disease

Rosaï-Dorfman-Destombes Disease

Destombes-Rosai-Dorfman Disease

Rosai-Dorfman-Destombes Disease

Slc29a3 Spectrum Disorder

Rosaï-Dorfman Disease

Sinus Histiocytosis

Slc29a3 Disorder

H Disease

Hlas

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Basic Information

Medical Symptom

Gene & Mutation

Related Drugs

Disease Model

References Literature

Histiocytosis-Lymphadenopathy Plus Syndrome, also known as h syndrome, is related to hemoglobin h disease and igg4-related disease, and has symptoms including fever An important gene associated with Histiocytosis-Lymphadenopathy Plus Syndrome is SLC29A3 (Solute Carrier Family 29 Member 3), and among its related pathways/superpathways are Nuclear events mediated by NFE2L2 and Circadian Clock. The drugs Prednisone and Mycophenolic acid have been mentioned in the context of this disorder. Affiliated tissues include heart and skin, and related phenotypes are delayed puberty and fever

Related ID：

MALACARDS：HST017

OMIM：602782

MESH：D015618