Lymph nodes - not lymphoma
Primary immunodeficiency disorders
Autoimmune lymphoproliferative syndrome




Topic Completed: 1 October 2014

Revised: 1 February 2019, last major update October 2014

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Autoimmune lymphoproliferative syndrome [title]

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Cite this page: DePond W. Autoimmune lymphoproliferative syndrome. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/lymphnodesALPS.html. Accessed April 23rd, 2019.
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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