Hemolytic Anemia and Lymphadenopathy

  • Svetlana O. Sharapova


Chronic or multi-lineage cytopenia should prompt enumeration of the double negative T cells (DNT) cells in peripheral blood and hence a suspicion of autoimmune lymphoproliferative syndrome (ALPS)

DNTs should be use in routine laboratory evaluation of ALPS patients together with the serum level of soluble Fas ligand, IL-10 and vitamin B12

Serum immunoglobulin levels help distinguish the ALPS due to FAS/FASL mutations from other subtypes

More than 90% of patients with FAS or FASL mutations exhibit hyper IgG and/or IgA


Autoimmune lymphoproliferative syndrome Double negative T cells Fas ligand 


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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Svetlana O. Sharapova
    • 1
  1. 1.Belarusian Research Center for Pediatric Oncology, Hematology and Immunology, Research Department, Immunology LaboratoryMinsk RegionBelarus

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