Cyclosporine A therapy for multisystem langerhans cell histiocytosis.
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.


METHODS
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, prednisolone, 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.
Minkov
M
M
St. Anna Children's Hospital, Vienna, Austria. minkov@ccri.univie.ac.at
Grois
N
N
Broadbent
V
V
Ceci
A
A
Jakobson
A
A
Ladisch
S
S
D000293:Adolescent; D000935:Antifungal Agents / Q000494:pharmacology / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D016572:Cyclosporine / Q000494:pharmacology / Q000627:therapeutic use*; D018450:Disease Progression; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D016896:Treatment Outcome
D000935:Antifungal Agents; D016572:Cyclosporine