Table 1.

Pertinent, brief clinical data and EBV status of 7 Taiwanese patients with HLH, for whom tissue or cells were available for analysis of the SAP/SH2D1A gene







Mutation in SAP
Case no.
Age, y
Sex
EBV status
Therapy (status)
Exon 2
Exon 3
1   7   M   +   IVIG (died)   None   None  
2   13   M   +   IVIG + VP-16 (died)   None   None  
3   21   F   -   IVIP + VP-16 (alive)   None   None  
4   24   M   +   Steroid + IVIG (alive)   None   None  
5   14   F   +   Steroid + VP-16 (died)   None   None  
6   15   M   +   IVIG + VP-16 (died)   None   None  
7
 
23
 
F
 
+
 
Steroid (alive)
 
None
 
None
 






Mutation in SAP
Case no.
Age, y
Sex
EBV status
Therapy (status)
Exon 2
Exon 3
1   7   M   +   IVIG (died)   None   None  
2   13   M   +   IVIG + VP-16 (died)   None   None  
3   21   F   -   IVIP + VP-16 (alive)   None   None  
4   24   M   +   Steroid + IVIG (alive)   None   None  
5   14   F   +   Steroid + VP-16 (died)   None   None  
6   15   M   +   IVIG + VP-16 (died)   None   None  
7
 
23
 
F
 
+
 
Steroid (alive)
 
None
 
None
 

IVIG indicates intravenous immunoglobulin; VP-16, etoposide.

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