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Lymphadenopathy and Splenomegaly

  • Joshua M. Dorn
  • Avni Y. Joshi
  • Roshini S. AbrahamEmail author
Chapter

Abstract

  • Autoimmune lymphoproliferative syndrome (ALPS) is characterized by chronic nonmalignant lymphoproliferation, splenomegaly, autoimmunity and increased risk of lymphoma

  • ALPS results from defects in the FAS pathway of cellular apoptosis, and most often from mutations in the FAS gene itself

  • Elevation of TCRαβ+CD45+CD3+CD4CD8 DNT cells is a characteristic feature of ALPS, and elevated soluble FAS ligand, vitamin B12, IL-10, and IL-18 can also be seen

  • Treatment is guided by severity of disease and Sirolimus has been shown to be successful in children with ALPS

Keywords

Direct antiglobulin test Autoimmune lymphoproliferative syndrome FAS 

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Joshua M. Dorn
    • 1
  • Avni Y. Joshi
    • 1
  • Roshini S. Abraham
    • 2
    Email author
  1. 1.Division of Allergic Diseases, Department of MedicineMayo ClinicRochesterUSA
  2. 2.Diagnostic Immunology Laboratory, Department of Pathology and Laboratory MedicineNationwide Children’s HospitalColumbusUSA

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