Autoimmune lymphoproliferative syndrome criteria: Difference between revisions
mNo edit summary |
|||
| Line 4: | Line 4: | ||
==Overview== | ==Overview== | ||
In 2009, an international consensus conference was held, and a revised set of diagnostic criteria was published in 2010 (Table 2) [16]. Based on this set of criteria, definitive diagnosis is based on the presence of two required criteria and one primary accessory criterion. A probable diagnosis is based on the presence of both required criteria plus one secondary accessory criterion. | |||
Following the international conference in 2009, a revised set of diagnostic criteria was published in 2010. According to this set of criteria, to reach a definitive diagnosis | |||
==Diagnostic Criteria== | ==Diagnostic Criteria== | ||
Revision as of 18:52, 30 June 2021
|
Autoimmune lymphoproliferative syndrome Microchapters |
|
Differentiating Autoimmune lymphoproliferative syndrome from other Diseases |
|---|
|
Diagnosis |
|
Treatment |
|
Case Studies |
|
Autoimmune lymphoproliferative syndrome criteria On the Web |
|
American Roentgen Ray Society Images of Autoimmune lymphoproliferative syndrome criteria |
|
Autoimmune lymphoproliferative syndrome criteria in the news |
|
Directions to Hospitals Treating Autoimmune lymphoproliferative syndrome |
|
Risk calculators and risk factors for Autoimmune lymphoproliferative syndrome criteria |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; David Teachey, MD [2]
Overview
In 2009, an international consensus conference was held, and a revised set of diagnostic criteria was published in 2010 (Table 2) [16]. Based on this set of criteria, definitive diagnosis is based on the presence of two required criteria and one primary accessory criterion. A probable diagnosis is based on the presence of both required criteria plus one secondary accessory criterion.
Following the international conference in 2009, a revised set of diagnostic criteria was published in 2010. According to this set of criteria, to reach a definitive diagnosis
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.