Haemophagocytic Lymphohistiocytosis in an Elderly Patient after Recent Cardiac Surgery

Table 2

Diagnostic criteria for haemophagocytic lymphohistiocytosis.

Diagnostic criterion	Case	Criteria score
1. A molecular diagnosis consistent with HLH	Positive	1
2. Fulfillment of five out of the eight criteria below:
Fever (>100.4°F, >38°C)	Present	1
Splenomegaly	Absent	0
Cytopenias affecting at least two of three lineages in the peripheral blood		1
Haemoglobin <9 g/100 ml	Present
Platelets <100 × 10^9/L	Present
Neutrophils <1 × 10^9/L	Absent
Hypertriglyceridemia and/or hypofibrinogenemia		1
Fasting triglyceride levels >3 mmol/liter	Present
Fibrinogen level <1.5 g/liter	Present
Ferritin ≥500 ng/ml	Present	1
Haemophagocytosis in bone marrow, central nervous system, spleen, or lymph nodes	Present	1
Low or absent natural killer cell activity	Not tested
Soluble CD25 (soluble IL-2 receptor) > 2400 U/ml	Not tested
Total		6/8

HLH-HaemophagocyticLymphohistiocytosis Criteria 1 and/or 2 must be present to make a diagnosis of HLH.