Table 1.

Overview of overall outcome in 113 patients with HLH treated according to the HLH-94 protocol

All patients (n = 113)Familial cases* (n = 25)
Alive after BMT 40 13  
Dead after BMT 25† 7† 
Alive without BMT 23‡  
Dead prior to BMT 25 5 
All patients (n = 113)Familial cases* (n = 25)
Alive after BMT 40 13  
Dead after BMT 25† 7† 
Alive without BMT 23‡  
Dead prior to BMT 25 5 
*

All these patients had an affected sibling with HLH.

†

One child, with a positive family history, died of a surgical hemorrhage unrelated to HLH.

‡

Three patients were still on therapy (2 on CSA alone; 1 also received corticosteroids); the others were off therapy for more than 12 months.

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