Results: Thirty-eight patients (15%) died during the follow-up period. Mean age of the decreased was 35.4 +/- 12.2 years (range 21-52 years) and the disease duration 8.6 +/- 8.2 years (range 0.6-20), the median length of the survival from the time of the diagnosis was 6.2 +/- 4.3 years.