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type 3 autoimmune lymphoproliferative syndrome - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

type 3 autoimmune lymphoproliferative syndrome

MONDO:8000023

Overview

A rare, primary immunodeficiency. It is caused by a currently undetermined defect in the Fas-induced apoptosis pathway. No mutations in Fas, FASLG or CASP10 are detectable. Disruption of Fas-induced apoptosis impairs lymphocyte homeostasis and immune tolerance. Characteristic laboratory findings include an increase in circulating, double-negative (CD4-/CD8-) T cells in the setting of immune-mediated anemia, thrombocytopenia and neutropenia. Clinical signs present in childhood include fatigue, pallor, bruising, hepatosplenomegaly and chronic, non-malignant, non-infectious lymphadenopathy. The clinical course is influenced by a strong association with other autoimmune disorders and an increased risk for developing Hodgkin and non-Hodgkin lymphoma.

ResearchLimited published research (4 articles)

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for type 3 autoimmune lymphoproliferative syndrome.

6 publications have been identified in PubMed for type 3 autoimmune lymphoproliferative syndrome. Kisho has analyzed 4 by research type. Research spans Review / Meta-Analysis (50%), Case Report / Case Series (25%), and Epidemiology / Natural History (25%).

Recent Publications

Rieux-Laucat F (2026). [PMID: 41997376](https://pubmed.ncbi.nlm.nih.gov/41997376/). *Immunol Lett*. [Review / Meta-Analysis]
Ding W (2025). [PMID: 40909280](https://pubmed.ncbi.nlm.nih.gov/40909280/). *Front Immunol*. [Case Report / Case Series]
Vázquez-Comendador JM (2025). [PMID: 39823212](https://pubmed.ncbi.nlm.nih.gov/39823212/). *J Clin Rheumatol*. [Epidemiology / Natural History]
Duong VT (2024). [PMID: 39469717](https://pubmed.ncbi.nlm.nih.gov/39469717/). *Front Immunol*. [Review / Meta-Analysis]

Data assembled from 2 of 12 sources · Last updated Sep 18, 2026, 6:51 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

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type 3 autoimmune lymphoproliferative syndrome

Overview

Research Landscape

Recent Publications

Medical References

Related Diseases

Questions for Your Care Team

Questions for Your Care Team