Skip to main content
Journal cover image

Autoimmune lymphoproliferative syndrome: an update and review of the literature.

Publication ,  Journal Article
Shah, S; Wu, E; Rao, VK; Tarrant, TK
Published in: Curr Allergy Asthma Rep
September 2014

Autoimmune lymphoproliferative syndrome (ALPS) is characterized by immune dysregulation due to a defect in lymphocyte apoptosis. The clinical manifestations may be noted in multiple family members and include lymphadenopathy, splenomegaly, increased risk of lymphoma, and autoimmune disease, which typically involves hematopoietic cell lines manifesting as multilineage cytopenias. Since the disease was first characterized in the early 1990s, there have been many advances in the diagnosis and management of this syndrome. The inherited genetic defect of many ALPS patients has involved (FAS) pathway signaling proteins, but there remain those patients who carry undefined genetic defects. Despite ALPS having historically been considered a primary immune defect presenting in early childhood, adult onset presentation is increasingly becoming recognized and more so in genetically undefined patients and those with somatic FAS mutations. Thus, future research may identify novel pathways and/or regulatory proteins important in lymphocyte activation and apoptosis.

Duke Scholars

Altmetric Attention Stats
Dimensions Citation Stats

Published In

Curr Allergy Asthma Rep

DOI

EISSN

1534-6315

Publication Date

September 2014

Volume

14

Issue

9

Start / End Page

462

Location

United States

Related Subject Headings

  • fas Receptor
  • T-Lymphocytes
  • Splenomegaly
  • Signal Transduction
  • Lymphocyte Activation
  • Immunosuppressive Agents
  • Humans
  • Diagnosis, Differential
  • Autoimmune Lymphoproliferative Syndrome
  • Apoptosis
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Shah, S., Wu, E., Rao, V. K., & Tarrant, T. K. (2014). Autoimmune lymphoproliferative syndrome: an update and review of the literature. Curr Allergy Asthma Rep, 14(9), 462. https://doi.org/10.1007/s11882-014-0462-4
Shah, Shaili, Eveline Wu, V Koneti Rao, and Teresa K. Tarrant. “Autoimmune lymphoproliferative syndrome: an update and review of the literature.Curr Allergy Asthma Rep 14, no. 9 (September 2014): 462. https://doi.org/10.1007/s11882-014-0462-4.
Shah S, Wu E, Rao VK, Tarrant TK. Autoimmune lymphoproliferative syndrome: an update and review of the literature. Curr Allergy Asthma Rep. 2014 Sep;14(9):462.
Shah, Shaili, et al. “Autoimmune lymphoproliferative syndrome: an update and review of the literature.Curr Allergy Asthma Rep, vol. 14, no. 9, Sept. 2014, p. 462. Pubmed, doi:10.1007/s11882-014-0462-4.
Shah S, Wu E, Rao VK, Tarrant TK. Autoimmune lymphoproliferative syndrome: an update and review of the literature. Curr Allergy Asthma Rep. 2014 Sep;14(9):462.
Journal cover image

Published In

Curr Allergy Asthma Rep

DOI

EISSN

1534-6315

Publication Date

September 2014

Volume

14

Issue

9

Start / End Page

462

Location

United States

Related Subject Headings

  • fas Receptor
  • T-Lymphocytes
  • Splenomegaly
  • Signal Transduction
  • Lymphocyte Activation
  • Immunosuppressive Agents
  • Humans
  • Diagnosis, Differential
  • Autoimmune Lymphoproliferative Syndrome
  • Apoptosis