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Table 2 HLH/MAS Evidence-Based Guideline Diagnostic Evaluation

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

Potential Laboratory Evaluation

 CBC w/ diff

 ESR

 Chem 10 (Na, K, Cl, CO2, BUN, Cr, Glucose, Ca, Mg, Phos)

 LFTs (AST, ALT, Tbili, Dbili)

 SPA Panel (IgG, IgM, IgA, C3, C4, CRP, Albumin, Protein)

 LDH

 Triglycerides

 Coagulation Studies (PT, PTT, INR, Fibrinogen, D-dimer)

 Infectious Studies (EBV PCR, CMV PCR, Blood Culture)

 CD107a Mobilization/NK Cell Degranulation

 IL-18

 CXCL9

 Soluble IL-2 Receptor

 Perforin/Granzyme Expression

 SAP/XIAP Expression (Males)

 Genetic Testing for FHL

Potential Radiologic Evaluation

 Chest X-ray

 Abdominal Ultrasound

  1. HLH hemophagocyticlymphohistiocytosis, MAS macrophage activation syndrome, SPA serum protein analysis panel, SAP SLAM-associated protein, XIAP X-linked inhibitor of apoptosis, FHL familial HLH
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Pediatric Rheumatology

ISSN: 1546-0096