Table 5.

Survival as reported in the 3 largest reports on HLH

PublicationYear of reportNumber of patientsSurvival
Janka (review)1 1983 121 5%  (1 yr)5-150 
Arico et al2 1996 122 22%  (5 yr)5-151 
Present study 2002 113 55%  (5 yr)5-151 
Present study (familial cases) 2002 25 51%  (5 yr)5-151 
PublicationYear of reportNumber of patientsSurvival
Janka (review)1 1983 121 5%  (1 yr)5-150 
Arico et al2 1996 122 22%  (5 yr)5-151 
Present study 2002 113 55%  (5 yr)5-151 
Present study (familial cases) 2002 25 51%  (5 yr)5-151 
F5-150

Of 121 patients reviewed, 5 of 101 with follow-up data survived more than 12 months.

F5-151

Probability of survival according to Kaplan-Meier estimate.

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