Primary immunodeficiency disorders

Autoimmune lymphoproliferative syndrome

Topic Completed: 1 October 2014

Minor changes: 3 June 2021

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PubMed Search: Autoimmune lymphoproliferative syndrome [title]

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Cite this page: DePond W. Autoimmune lymphoproliferative syndrome. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphnodesALPS.html. Accessed September 20th, 2021.
Definition / general
Case reports
Microscopic (histologic) description
  • Paracortical hyperplasia, expanded interfollicular areas; polyclonal plasmacytosis (Am J Pathol 1998;153:1541)
  • Frequent mitotic figures
  • Often florid follicular hyperplasia with focal progressive transformation of germinal centers
  • Occasional follicular involution
Positive stains
  • T cells - CD3, CD57, usually alpha/beta T cell receptor; also perforin, TIA1, CD57
Negative stains
Molecular / cytogenetics description
  • Mutation in death domain of FAS gene
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