Table 4 Modified diagnostic criteria
From: Macrophage activation syndrome in MDA5 antibody-positive dermatomyositis and COVID-19 infection
1 | Molecular diagnosis of hemophagocytic lymphohistiocytosis (HLH) or X-linked lymphoproliferative syndrome (XLP) |
2 | Or at least 3 of 4 of the following Fever Splenomegaly Cytopenias (minimum 2 cell lines reduced) Hepatitis |
3 | And at least 1 of 4 of the following Hemophagocytosis ↑ Ferritin ↑ sIL2Rα (age based) Absent or very decreased NK function |
4 | Other results supportive of HLH diagnosis Hypertriglyceridemia Hypofibrinogenemia Hyponatremia |