Table 1

Current ALPS diagnostic and study inclusion/exclusion criteria

Current diagnostic criteria for ALPS
Chronic nonmalignant lymphoproliferation 
Elevated peripheral blood DNTs 
Defective in vitro Fas-mediated apoptosis 
            MUST MEET ALL THREE CRITERIA FOR DIAGNOSIS* 
Supportive but not diagnostic: 
    Genetic mutation (FAS, FASL, CASP10, NRAS
    Autoimmunity 
    Family history 
    Histopathology 
Current diagnostic criteria for ALPS
Chronic nonmalignant lymphoproliferation 
Elevated peripheral blood DNTs 
Defective in vitro Fas-mediated apoptosis 
            MUST MEET ALL THREE CRITERIA FOR DIAGNOSIS* 
Supportive but not diagnostic: 
    Genetic mutation (FAS, FASL, CASP10, NRAS
    Autoimmunity 
    Family history 
    Histopathology 
Study inclusion and exclusion criteria
Inclusion criteria 
    Age 1-21 y 
    Diagnosis of Evans syndrome by treating physician 
    Documented autoimmune cytopenias 
        Either 2 or 3 cell types (RBCs, WBC, platelets), or 
        Autoimmune thrombocytopenia and strongly positive direct antiglobulin test 
Exclusion criteria 
    Autoimmune disease 
        Systemic lupus erythematosus 
        Juvenile idiopathic arthritis 
    Malignancy 
    Immunosuppression after solid organ transplantation 
    Concurrent immunomodulating medications, except for low-dose corticosteroids (≤ 0.5 mg/kg/d) 
Study inclusion and exclusion criteria
Inclusion criteria 
    Age 1-21 y 
    Diagnosis of Evans syndrome by treating physician 
    Documented autoimmune cytopenias 
        Either 2 or 3 cell types (RBCs, WBC, platelets), or 
        Autoimmune thrombocytopenia and strongly positive direct antiglobulin test 
Exclusion criteria 
    Autoimmune disease 
        Systemic lupus erythematosus 
        Juvenile idiopathic arthritis 
    Malignancy 
    Immunosuppression after solid organ transplantation 
    Concurrent immunomodulating medications, except for low-dose corticosteroids (≤ 0.5 mg/kg/d) 

Data are from Bleesing et al, Oliveira and Gupta, and Oliveira et al with permission.

*

Except ALPS types 1b and 1s, which have negative apoptosis assays.

Low-dose corticosteroids have been shown not to affect results.

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