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.