Using a Minimal Parameter Set for Early Diagnosis of Hemophagocytic Lymphohistiocytosis in Non-European Children

26 January 2022: Articles

Challenging differential diagnosis

Yu Nakashima ^B , Hiroshi Koga ^A*

DOI: 10.12659/AJCR.934037

Am J Case Rep 2022; 23:e934037

Table 1. Diagnostic assessment of HLH-2004 criteria and the minimal parameter set in the cases of 2 infants with HLH.

	Case 1 (Japanese neonate)		Case 2 (African infant)
	Our center	Tertiary center	Our center	Tertiary center
Age on admission (days)	4	4	39	41
HLH-2004 diagnostic criteria	Not fulfilled	Fulfilled	Fulfilled	Fulfilled
Fever	Present	Present	Present	Present
Splenomegaly	Present	Present	Present	Present
Cytopenias in ≥2 lineages in peripheral blood	Present	Present	Present	Present
Hyperferritinemia ≥500 μg/L	Present	Present	Present	Present
Soluble CD25 ≥2400 U/mL	Not evaluated	Absent	Present	Present
Hypertriglyceridemia ≥265 mg/dL and/or hypofibrinogenemia ≤1.5 g/L	Absent	Present	Present	Present
Hemophagocytosis in tissue biopsy	Not evaluated	Absent	Not evaluated	Present
Low or absent NK-cell activity	Not evaluated	Present	Not evaluated	Present
Minimal parameter set	Fulfilled	Fulfilled	Fulfilled	Fulfilled
Major criteria
Splenomegaly	Present	Present	Present	Present
Hemophagocytosis in tissue biopsy	Not evaluated	Absent	Not evaluated	Present
Minor criteria
Cytopenias in ≥2 lineages in peripheral blood	Present	Present	Present	Present
Hyperferritinemia ≥1000 μg/L	Present	Present	Absent	Present
Hypertriglyceridemia ≥265 mg/dL and/or hypofibrinogenemia ≤1.5 g/L	Absent	Present	Present	Present
HLH – hemophagocytic lymphohistiocytosis; ^* A diagnosis of HLH can be established if ≥5 of 8 criteria are fulfilled.; ^** A diagnosis of HLH can be established if 2 major criteria, 1 major and ≥1 minor criteria, or 3 minor criteria are fulfilled.