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Descriptor English: Autoimmune Lymphoproliferative Syndrome
Descriptor Spanish: Síndrome Linfoproliferativo Autoinmune
Descriptor síndrome linfoproliferativo autoinmunitario
Entry term(s) deficiencia de caspasa 8
deficiencia de caspasa-8
síndrome de Canale-Smith
síndrome linfoproliferativo autoinmunitario tipo 1 autosómico dominante
síndrome linfoproliferativo autoinmunitario tipo 2B
síndrome linfoproliferativo autoinmunitario tipo 2B (ALPS2B)
Scope note: Raro trastorno congénito del tejido linfático debido a mutaciones en ciertos genes de la vía del ligando Fas-Fas. Las causas conocidas incluyen mutaciones en los genes de las proteínas FAS, TNFSF6, ANR, CASP8 y CASP10. Las características clínicas incluyen LINFADENOPATÍA,  ESPLENOMEGALIA y AUTOINMUNIDAD. 
Descriptor Portuguese: Síndrome Linfoproliferativa Autoimune
Descriptor French: Syndrome lymphoprolifératif avec auto-immunité
Entry term(s): Autoimmune Lymphoproliferative Syndrome Type 1, Autosomal Dominant
Autoimmune Lymphoproliferative Syndrome Type 2B
Autoimmune Lymphoproliferative Syndrome Type 2B (ALPS2B)
Autoimmune Lymphoproliferative Syndrome, Type I, Autosomal Dominant
Autoimmune Lymphoproliferative Syndrome, Type IIb
Autoimmune Lymphoproliferative Syndromes
Canale Smith Syndrome
Canale-Smith Syndrome
Canale-Smith Syndromes
Caspase 8 Deficiencies
Caspase 8 Deficiency
Caspase-8 Deficiencies
Caspase-8 Deficiency
Deficiencies, Caspase 8
Deficiencies, Caspase-8
Deficiency, Caspase 8
Deficiency, Caspase-8
Lymphoproliferative Syndrome, Autoimmune
Lymphoproliferative Syndromes, Autoimmune
Syndrome, Autoimmune Lymphoproliferative
Syndrome, Canale Smith
Syndrome, Canale-Smith
Syndromes, Autoimmune Lymphoproliferative
Syndromes, Canale-Smith
Tree number(s): C15.604.515.138
C16.320.089
C20.111.288
C20.683.515.124
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D056735
Scope note: Rare congenital lymphoid disorder due to mutations in certain Fas-Fas ligand pathway genes. Known causes include mutations in FAS, TNFSF6, NRAS, CASP8, and CASP10 proteins. Clinical features include LYMPHADENOPATHY; SPLENOMEGALY; and AUTOIMMUNITY.
Allowable Qualifiers: BL blood
CF cerebrospinal fluid
CI chemically induced
CL classification
CO complications
DG diagnostic imaging
DH diet therapy
DI diagnosis
DT drug therapy
EC economics
EH ethnology
EM embryology
EN enzymology
EP epidemiology
ET etiology
GE genetics
HI history
IM immunology
ME metabolism
MI microbiology
MO mortality
NU nursing
PA pathology
PC prevention & control
PP physiopathology
PS parasitology
PX psychology
RH rehabilitation
RT radiotherapy
SU surgery
TH therapy
UR urine
VE veterinary
VI virology
Previous Indexing: Autoimmune Diseases (1997-2009)
Lymphoproliferative Disorders (1983-2009)
Public MeSH Note: 2010
History Note: 2010
Related: Caspase 10 MeSH
Caspase 8 MeSH
Glucocorticoid-Induced TNFR-Related Protein MeSH
fas Receptor MeSH
DeCS ID: 53551
Unique ID: D056735
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 2010/01/01
Date of Entry: 2009/07/06
Revision Date: 2017/07/17
Autoimmune Lymphoproliferative Syndrome - Preferred
Concept UI M0528916
Scope note Rare congenital lymphoid disorder due to mutations in certain Fas-Fas ligand pathway genes. Known causes include mutations in FAS, TNFSF6, NRAS, CASP8, and CASP10 proteins. Clinical features include LYMPHADENOPATHY; SPLENOMEGALY; and AUTOIMMUNITY.
Preferred term Autoimmune Lymphoproliferative Syndrome
Entry term(s) Autoimmune Lymphoproliferative Syndrome, Type I, Autosomal Dominant
Autoimmune Lymphoproliferative Syndromes
Canale Smith Syndrome
Canale-Smith Syndrome
Canale-Smith Syndromes
Lymphoproliferative Syndrome, Autoimmune
Lymphoproliferative Syndromes, Autoimmune
Syndrome, Autoimmune Lymphoproliferative
Syndrome, Canale Smith
Syndrome, Canale-Smith
Syndromes, Autoimmune Lymphoproliferative
Syndromes, Canale-Smith
Autoimmune Lymphoproliferative Syndrome Type 1, Autosomal Dominant - Narrower
Concept UI M0529217
Preferred term Autoimmune Lymphoproliferative Syndrome Type 1, Autosomal Dominant
Autoimmune Lymphoproliferative Syndrome Type 2B - Narrower
Concept UI M0528917
Scope note Autoimmune lymphoproliferative syndrome due to mutations in CASPASE 8 gene.
Preferred term Autoimmune Lymphoproliferative Syndrome Type 2B
Entry term(s) Autoimmune Lymphoproliferative Syndrome Type 2B (ALPS2B)
Autoimmune Lymphoproliferative Syndrome, Type IIb
Caspase 8 Deficiencies
Caspase 8 Deficiency
Caspase-8 Deficiencies
Caspase-8 Deficiency
Deficiencies, Caspase 8
Deficiencies, Caspase-8
Deficiency, Caspase 8
Deficiency, Caspase-8



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