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Table 1 HLH/MAS Evidence-Based Guideline Entry Criteria

From: Calm in the midst of cytokine storm: a collaborative approach to the diagnosis and treatment of hemophagocytic lymphohistiocytosis and macrophage activation syndrome

Clinical and Laboratory Criteria

1. Fever (high, unremitting)

2. Ferritin (≥500 ng/mL)

3. Other Considerations

 Rheumatologic/hematologic/immunologic conditions that predispose to HLH/MASa

 Neurologic symptomsb

 HSM

 Cytopeniasc

 Hepatobiliary dysfunctiond

 DIC

 EBV or other viral infection

  1. HLH hemophagocyticlymphohistiocytosis, MAS macrophage activation syndrome, HSM hepatosplenomegaly, DIC disseminated intravascular coagulation, EBV Epstein-Barr virus
  2. aIncluding but not limited to systemic juvenile idiopathic arthritis, systemic lupus erythematosus, Kawasaki Disease, familial HLH, lymphoma, Chediak-Higashi Syndrome, Griscelli Syndrome, Hermansky-Pudlak Syndrome type 2, X-linked lymphoproliferative disease 1 & 2
  3. bHeadaches, cognitive changes, focal examination findings, seizures, findings not explained by degree of illness/medications
  4. cHemoglobin < 9 g/dL, platelets < 200 109/L, absolute neutrophil count < 1000/mm3
  5. dElevated liver function tests or bilirubin
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Pediatric Rheumatology

ISSN: 1546-0096