Prednisoneb Vinblastinec ±Methotrexated
6-MPe Prednisone/ Vinblastineg ±Methotrexateh
Same as group 1
Not yet established whether results with 12 months of therapy are better than 6 months
Same as group 1
Same as group 2
6 months only
"Patients on systemic chemotherapy should receive standard supportive care including sulfamethoxazole/ trimethoprim (5 mg/kg/day of trimethoprim) in 2 divided doses per day for 3 days per week (sulfamethoxa-zole/trimethoprim should not be administered during methotrexate administration).
6Oral prednisone 40 mg/m2 daily in 3 divided doses as a 4-week course, followed by a tapering dose over a period of 2 weeks. Poor responders should receive a further 6-week course of prednisone 40 mg/m2 daily on days 1-3 of each week for an additional 6 weeks.
"Vinblastine 6 mg/m2 IV bolus on day 1 of weeks 1, 2, 3, 4, 5, and 6. This 6-week course of vinblastine is repeated for poor responders.
dMethotrexate 500 mg/m2 24 hours—infusion with folinic acid (leucovorin) rescue day 1 of weeks 1, 3, and 5. Ten percent of the dose is given as IV bolus over 30 minutes, followed by 90% of the dose as a 23.5-hour infusion with 2000 mL/m2 hydration.
Folinic acid 12 mg/m2 is given 24 and 30 hours after methotrexate infusion is completed (at 48 and 54 hours after methotrexate therapy is started).
It has not been established whether the addition of methotrexate improves results.
eOral 6-mercaptopurine (6-MP) 50 mg/m2 daily until the end of the 12th month from commencement of therapy.
-^Pulses of oral prednisone 40 mg/m2 daily in 3 doses on days 1-5 every 3 weeks, starting on day 1 of week 7 in patients who have no active disease after course 1 or on day 1 of week 13 in patients who have no active disease or the active disease is better after course 2, continued until the end of month 12.
^Vinblastine 6 mg/m2/day IV bolus once weekly for 3 weeks, starting on day 1 of week 7 in patients with no active disease after course 1 or on day 1 of week 13 in patients with no active disease or the active disease is better after course 2, continued until the end of month 12.
hMethotrexate 20 mg/m2 orally, once weekly, until the end of month 12. It has not been established whether the addition of methotrexate improves results.
after a period of remission, often respond well to the drugs with which they were initially treated. A number of chemotherapeutic approaches have had limited success. There is anecdotal experience using tumor necrosis factor (TNF) inhibitors in recurrent or refractory disease as well as pamidronate for bone lesions. Several studies, however, have demonstrated significant activity to 2-chlorodeoxyadenosine (2-CdA)
in recurrent and refractory LCH. In addition, the combination of 2-CdA and highdose cytosine arabinoside (Ara-C) has been used in refractory patients. Our current approach is to treat refractory patients with two courses of 2-CdA (6 mg/m2/day in 50 mL normal saline over 2 hours for 5 consecutive days every 4 weeks). If there is a good response, then the patient receives 2-4 additional courses of 2-CdA. If there is a poor initial response after two courses of 2-CdA, Ara-C (100 mg/kg in 75 mL normal saline every 12 hours for 4 doses to begin on first day of 2-CdA administration) is added to two to four more courses of the 2-CdA. Stem cell transplantation for high-risk or refractory patients warrants further evaluation.
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