Comparison of 2 sets of diagnostic criteria that can be applied to patients with suspected MAS
| . | HLH-200412 (5 of 8 needed) . | EULAR/ACR/PRINTOMAS/SJIA (5 of 7 needed; must include the elements in italics) . |
|---|---|---|
| Clinical | Known or suspected SJIA | |
| Constitutional findings | Fever | Fever |
| Physical examination and imaging findings | Splenomegaly | |
| Peripheral blood counts | Cytopenias affecting 2 or 3 lineages: Hemoglobin <9 g/dL Platelets <100 × 109/L Neutrophils <1.0 × 109/L | Platelet count ≤181 × 109/L |
| Other laboratory values | Ferritin ≥500 ng/mL Fasting triglycerides ≥265 mg/dL and/or fibrinogen ≤1.5 g/L Soluble CD25 ≥2400 U/mL Low or absent natural killer cell activity | Ferritin >684 ng/mL Triglycerides >156 mg/dL Fibrinogen ≤360 mg/dL Aspartate aminotransferase >48 U/L |
| Pathology results | Hemophagocytosis in the bone marrow, spleen, or lymph nodes |
| . | HLH-200412 (5 of 8 needed) . | EULAR/ACR/PRINTOMAS/SJIA (5 of 7 needed; must include the elements in italics) . |
|---|---|---|
| Clinical | Known or suspected SJIA | |
| Constitutional findings | Fever | Fever |
| Physical examination and imaging findings | Splenomegaly | |
| Peripheral blood counts | Cytopenias affecting 2 or 3 lineages: Hemoglobin <9 g/dL Platelets <100 × 109/L Neutrophils <1.0 × 109/L | Platelet count ≤181 × 109/L |
| Other laboratory values | Ferritin ≥500 ng/mL Fasting triglycerides ≥265 mg/dL and/or fibrinogen ≤1.5 g/L Soluble CD25 ≥2400 U/mL Low or absent natural killer cell activity | Ferritin >684 ng/mL Triglycerides >156 mg/dL Fibrinogen ≤360 mg/dL Aspartate aminotransferase >48 U/L |
| Pathology results | Hemophagocytosis in the bone marrow, spleen, or lymph nodes |
ACR, American College of Rheumatology; EULAR, European League Against Rheumatism. PRINTO, Paediatric Rheumatology International Trials Organisation.