. Author manuscript; available in PMC: 2018 Oct 1.

Published in final edited form as: Pediatr Clin North Am. 2017 Aug 18;64(5):1071–1088. doi: 10.1016/j.pcl.2017.06.007

Table 1.

Five inflammation pathobiology phenotypes and putative adjunctive therapies

Phenotype	Clinical Criteria	Biomarker/Prototype	Adjunctive Therapy
Thrombocytopenia Associated MOF	Platelets < 100,000/mm³ Acute Kidney Injury Elevated LDH	ADAMTS 13< 57% Discussed Prototypes = Purpura fulminans/Atypical HUS	a) Plasma Exchange^{9,,11–15,61} removes ultra large vWF multimers and restores ADAMTS13 activity b) C5a Antibody^16–19 Inhibits activated complement (FDA approved for aHUS)
Immune paralysis Associated MOF	Persistent or Secondary Infections	Monocyte HLA-DR expression < 30% or 8,000 molecules; Whole blood ex vivo TNF response to LPS < 200 pg/mL; Absolute Lymphocyte Count < 1,000 mm³ Discussed Prototype = H1N1/MRSA	GM-CSF^25,29,30 Immune suppressant withdrawal²⁸ Restores TNF response to endotoxin
Hyperleukocytosis and pulmonary hypertension associated MOF	Age < 6 months Pulmonary HTN	WBC > 50,000 mm³ Discussed Prototype = Critical Pertussis	Extracorporeal Leukoreduction³⁶ removes circulating WBC and decreases pulmonary hypertension
Sequential MOF with liver failure	Respiratory distress Followed by Hepatobiliary Dysfunction	s-FasL > 200 pg/mL Discussed Prototype = Epstein Barr Virus Lymphoproliferative Disease	a) Hold immune suppressants b) Give anti CD20 monoclonal antibody^44,45 removes EBV reservoir (FDA approved for PTLD)
Macrophage Activation Syndrome	Hepatobiliary Dysfunction and Disseminated Intravascular Coagulation	Ferritin > 500 ng/mL Discussed Prototype = Viral Hemorrhagic Fevers	IVIG + Steroids + Plasma Exchange⁶¹ Anakinra^48,57 Tocilizumab^58,59 decreases macrophage inflammation