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Autoimmune Lymphoproliferative Syndrome

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.
Also Known As:
Autoimmune Lymphoproliferative Syndrome, Type I, Autosomal Dominant; Autoimmune Lymphoproliferative Syndrome, Type IIb; Canale-Smith Syndrome; Autoimmune Lymphoproliferative Syndromes; Canale-Smith Syndromes; Caspase 8 Deficiencies; Caspase-8 Deficiencies; 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; Autoimmune Lymphoproliferative Syndrome Type 1, Autosomal Dominant; Autoimmune Lymphoproliferative Syndrome Type 2B; Autoimmune Lymphoproliferative Syndrome Type 2B (ALPS2B); Canale Smith Syndrome; Caspase 8 Deficiency; Caspase-8 Deficiency
Networked: 223 relevant articles (7 outcomes, 8 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Cytopenia
2. Lymphadenopathy
3. Splenomegaly
4. Autoimmune Diseases (Autoimmune Disease)
5. Autoimmune Lymphoproliferative Syndrome

Experts

1. Rao, V Koneti: 17 articles (01/2020 - 05/2005)
2. Dale, Janet K: 13 articles (04/2012 - 07/2002)
3. Straus, Stephen E: 12 articles (04/2009 - 07/2002)
4. Rieux-Laucat, Frédéric: 11 articles (01/2018 - 06/2003)
5. Puck, Jennifer M: 11 articles (03/2014 - 07/2002)
6. Price, Susan: 9 articles (01/2020 - 07/2009)
7. Davis, Joie: 9 articles (03/2014 - 09/2002)
8. Fleisher, Thomas A: 9 articles (03/2014 - 07/2002)
9. Lenardo, Michael J: 8 articles (01/2020 - 09/2002)
10. Fischer, Alain: 8 articles (01/2018 - 06/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Autoimmune Lymphoproliferative Syndrome:
1. Sirolimus (Rapamycin)FDA Link
2. Death Domain ReceptorsIBA
3. Caspase 10IBA
4. 2,6-dinitrotoluene (DNT)IBA
5. Caspase 8 (Caspase-8)IBA
6. CaspasesIBA
7. Interleukin-2 Receptors (IL 2 Receptor)IBA
8. pyrimethamine drug combination fanasil (Fansidar)FDA Link
9. Small Interfering RNA (siRNA)IBA
10. Proliferating Cell Nuclear Antigen (PCNA)IBA

Therapies and Procedures

1. Therapeutics
2. Ligation
3. Splenectomy
4. Stem Cell Transplantation
5. Liver Transplantation