Permanent Consequences in Langerhans Cell Histiocytosis Single Center Study

Introduction: Langerhans cell Histiocytosis (LCH) is a rare disease characterized by clonal prolifera-tion of Histiocytosis in different tissues. Permanent consequences (PC) described among subjects with Langerhans cell histiocytosis (LCH).In this study we report the prevalence of permanent sequel among long – term survivors of LCH in our center. Methods: We had 30 cases of LCH from 1989 – 2001 who came for at least 3 years after diagnosis. In-formation has been collected from their disease history, and on type and date of onset of any PC. The cumulative risks of developing a PC have been calculated from the date of LCH diagnosis using the Kaplan-Meier and non-parametric method. Results: Among 30 patients 53.3% were female, 46.67% male, mean age at diagnosis 56.86%±7.79 months (range 7-156), median 42.5 months. 19 (63.33%) had single system (SS) and 11 (36.66%) mul-tisystem (MS). Mean age at SS 5.97 ±1.03 yr, mean age of MS 7.59±1.05 yr. Mean age at follow up 11.3 ± 0.9 yr, median 11.5 yr, range 4.16-22 yr. Mean duration of follow up 6.57 ± 0.76 yr, median 5 yr, and range 3-18 yr. Nine of 30 cases (30%) had at least 1PC; in SS (26.3 %) and in MS (36.7%). The most frequent PC was diabetes insipidus (DI) 16.7%, in SS 5.26% , in MS 36.36%, the difference is significant P