Chemotherapy:
Standard chemotherapy regimen for high-risk LCH as per the LCH IV protocol
Induction phase consists of six weeks with Vinblastine (6mg/m2) once a week for 6 weeks and Prednisolone (40mg/m2/day) daily for 4 weeks taper over the next 2 weeks. At the end of 6 weeks FDG-PET scan is done to look for remission. If FDG-PET negative (remission)patient would be started on maintenance therapy. Non-responders would receive another 6 week cycle of the same medication. If still remission not achievable, second-line chemotherapeutic agents were used.
The maintenance phase consists of Vinblastine (6mg/m2) given once in three weeks along with prednisolone 40mg/m2/day for 5 days in the same week for one year period.
Modified low dose induction regimen
Modified induction regimen of low dose Cytarabine (100mg/m2) every 3 weeks along with Prednisolone (40mg/m2/day) daily for 4 weeks tapered over the next 2 weeks ( one cycle consists of 6 weeks).
Maintenance chemotherapy includes continuation of Cytarabine (100mg/m2) every 3 weeks and prednisolone 40mg/m2/day for 5 days in the same week for 1 year was instituted if FDG-PET at 6 weeks showed disease remission.
Liver Transplantation: A FDG-PET documented disease remission was always mandatory prior to a LT. LT was offered for acute decompensation or patients with compensated cirrhosis and sclerosing cholangitis with portal hypertension. Patients who did not tolerate the full course of chemotherapy were also considered for a LT during mid-cycle. Where possible, LT was planned to coincide with the completion of chemotherapy. To avoid the systemic effects of chemotherapy, a 2-week weaning off period was given between the last cycle of chemotherapy and LT. Post-LT, chemotherapy was recommenced after 3 weeks.
Immunosuppression: Post LT, all patients were started on steroid and tacrolimus-based immunosuppression targeting a trough levels of 8-10 ng/dL in the first 3-4 weeks post-LT. The levels are then adjusted to target a trough level of 5-6ng/dL once chemotherapy is restarted (usually 3 weeks post-LT). Trough levels at 3-4ng/dL were maintained after an year of transplant. Low dose steroids (1mg for children under 4 years and 2mg for those over 4 years) were continued as per the institute protocol.
Follow up :
A PET scan was done
1) At the end of chemotherapy to confirm disease remission.
2) On follow-up if there is clinical or biochemical suspicion of disease recurrence.
Periodic monitoring of tacrolimus trough levels and of graft function (with LFT and hepatic doppler)was done in the follow up period.