Table 1.

Clinical and Biological Status of 17 Patients With EBV-HLH

Sex  4M, 13F  
Median age (range) at diagnosis  4 yr (0.8-12.3)  
Family history of HLH  None  
Initial clinical presentation (no. of cases)  
 Persistent fever (≥38.5°C)  17 
 Hepatosplenomegaly  15  
 Coagulopathy  13  
 CNS symptoms  3  
 Lymphadenopathy  10  
 Jaundice  
 Pleural effusion  6  
Initial biological presentation (no. of cases)* 
 Cytopenia  17  
 Hypertriglyceridemia  12 
 Hypofibrinogenemia  13  
 Serum ferritin >1,000 ng/mL 12  
 Serum LDH >1,000 IU/L  16  
 Specimen positive for EBV-DNA  17 
Sex  4M, 13F  
Median age (range) at diagnosis  4 yr (0.8-12.3)  
Family history of HLH  None  
Initial clinical presentation (no. of cases)  
 Persistent fever (≥38.5°C)  17 
 Hepatosplenomegaly  15  
 Coagulopathy  13  
 CNS symptoms  3  
 Lymphadenopathy  10  
 Jaundice  
 Pleural effusion  6  
Initial biological presentation (no. of cases)* 
 Cytopenia  17  
 Hypertriglyceridemia  12 
 Hypofibrinogenemia  13  
 Serum ferritin >1,000 ng/mL 12  
 Serum LDH >1,000 IU/L  16  
 Specimen positive for EBV-DNA  17 

Abbreviations: EBV-HLH, Epstein-Barr virus–associated hemophagocytic lymphohistiocytosis; LDH, lactate dehydrogenase.

*

Normal laboratory values are reported in the text.

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