Cyclosporine A therapy for multisystem Langerhans cell histiocytosis

✍ Scribed by Minkov, M.; Grois, N.; Broadbent, V.; Ceci, A.; Jakobson, A.; Ladisch, S.

• Publisher: John Wiley and Sons
• Year: 1999
• Tongue: English
• Weight: 59 KB
• Volume: 33
• Category: Article
• ISSN: 0098-1532
• DOI: 10.1002/(sici)1096-911x(199911)33:5<482::aid-mpo8>3.0.co;2-y

✦ Synopsis

Background. Treatment of multisystem Langerhans cell histiocytosis (LCH) remains difficult. Various regimens of single and multiagent chemotherapy have been used, but a significant proportion of patients fail to respond to treatment. Procedure. We have evaluated the use of cyclosporine A (CSA) in a controlled group of patients, who had received a systematic primary therapy (LCH-I). Patients received CSA either as a single agent (10 patients) or in combination with vinblastine, etoposide, pred-nisolone, and/or antithymocyte globulin (16 patients). Results. Among the total of 26 patients treated, a single patient developed a complete response and three a partial response, whereas 85% (22 patients) had no response to CSA. Conclusions. CSA is at best of limited value in the treatment of patients with multisystem LCH, particularly those who had progressive disease while receiving chemotherapy.