Autoimmune lymphoproliferative syndrome criteria: Difference between revisions
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{{CMG}}; David Teachey, MD [mailto:TEACHEYD@email.chop.edu] | |||
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Revision as of 17:41, 20 June 2016
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; David Teachey, MD [2]
Overview
Diagnostic Criteria
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
- 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
- Elevated biomarkers
- 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
References
- ↑ 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.