Current ALPS diagnostic and study inclusion/exclusion criteria
Current diagnostic criteria for ALPS . |
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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 . |
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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 . |
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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 . |
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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)† |