Autoimmune lymphoproliferative syndrome criteria

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Editor-In-Chief: David Teachey, MD [1]

Overview

Old criteria^[1]

Required
- Chronic non-malignant lymphoproliferation
- Elevated peripheral blood Double Negative T cells (DNTs)
- Defective in vitro Fas mediated apoptosis

New criteria^[2]

Required
- Chronic non-malignant lymphoproliferation (>6 months lymphadenopathy and/or splenomegaly)
- Elevated peripheral blood DNTs
Accessory
- Primary Accessory
  - Defective in vitro Fas mediated apoptosis
  - Somatic or germline mutation in ALPS causative gene (FAS, FASL, CASP10)
- Secondary Accessory
  - Elevated biomarkers
    - Plasma sFASL >200pg/ml
    - Plasma IL-10 >20pg/ml
    - Plasma or serum vitamin B12 >1500ng/L
    - Plasma IL-18 >500pg/ml
  - Immunohistochemical findings on biopsy consistent with ALPS as determined by experienced hematopathologist
  - Autoimmune cytopenias and polyclonal hypergammaglobulinemia
  - Family history of ALPS or non-malignant lymphoproliferation
Definitive diagnosis: Required plus one primary accessory criteria
Probable diagnosis: Required plus one secondary accessory criteria
Definitive and Probable ALPS should be TREATED THE SAME and patients counseled that they have ALPS if definitive or probable

↑ Sneller MC, Dale JK, Straus SE (2003). "Autoimmune lymphoproliferative syndrome". Curr Opin Rheumatol. 15 (4): 417–21. PMID 12819469.
↑ Oliveira JB, Bleesing JJ, Dianzani U, Fleisher TA, Jaffe ES, Lenardo MJ; et al. (2010). "Revised diagnostic criteria and classification for the autoimmune lymphoproliferative syndrome (ALPS): report from the 2009 NIH International Workshop". Blood. 116 (14): e35–40. doi:10.1182/blood-2010-04-280347. PMC 2953894. PMID 20538792.