Table 4

Distribution of clinical and laboratory abnormalities in patients with HLH

AbnormalitiesN% [90% confidence interval]
Fever 18/19 95 [77-100] 
Splenomegaly 16/18 89 [69-98] 
Cytopenia more than 1 cell line 19/19 100 [85-100] 
Anemia 14/19 74 [52-89] 
Thrombocytopenia 19/19 100 [85-100] 
Neutropenia 19/19 100 [85-100] 
Hypertriglyceridemia 14/18 78 [56-92] 
Hypofibrinogenemia 10/19 53 [32-73] 
sIL2-R elevation 10/10 100 [74-100] 
Low natural killer cell function 0/1 0 [0-95] 
Hemophagocytosis 12/16 75 [52-91] 
AbnormalitiesN% [90% confidence interval]
Fever 18/19 95 [77-100] 
Splenomegaly 16/18 89 [69-98] 
Cytopenia more than 1 cell line 19/19 100 [85-100] 
Anemia 14/19 74 [52-89] 
Thrombocytopenia 19/19 100 [85-100] 
Neutropenia 19/19 100 [85-100] 
Hypertriglyceridemia 14/18 78 [56-92] 
Hypofibrinogenemia 10/19 53 [32-73] 
sIL2-R elevation 10/10 100 [74-100] 
Low natural killer cell function 0/1 0 [0-95] 
Hemophagocytosis 12/16 75 [52-91] 
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