Presentation of hemophagocytic lymphohistiocytosis due to a novel MUNC 13–4 mutation masked by partial therapeutic immunosuppression

Table 2 Laboratory values meeting HLH diagnostic criteria found prior to, during and after trial of anakinra

HLH diagnostic criteria		Patient laboratory values
Timing relative to anakinra trial		Admission to prior to anakinra	During anakinra	One week after anakinra
Cytopenia in ≥ 2 cell lines
	Anemia	No	Yes	No
	Thrombocytopenia	Yes	Yes	Yes
	Neutropenia	Yes	Yes	Yes
Fasting Hypertriglyceridemia		-	Yes	Yes
Hypofibrinogenemia		Yes	No	-
Hyperferritinemia		Yes	Yes	Yes
Elevated sCD25		-	Yes	-
NK cell activity
	LU₂₀ NK	reduced	markedly reduced	-
	CD107a	-	Yes	-
Hemophagocytosis in bone marrow		some	-	numerous

ISSN: 1546-0096