The objective of this study is to determine the incidence of complete and partial response
and the duration of response in patients with Langerhans Cell Histiocytosis (LCH) treated
with sequential administration of oral 6-Thioguanine (6-TG) after Methotrexate (MTX).
- Patients with histologic proof of LCH who have multifocal or multisystem disease
- Patients must have a life expectancy of at least 8 weeks.
- All patients must have ECOG performance level rating of-< 2.
- Patients or their parents (guardian) must sign an informed consent indicating that
they are aware of the investigational nature of the study, using commercially
- Patients must have recovered from the toxic effects of prior therapy before entering
this study or at least 2 weeks should have elapsed since the end of last course of
- Patients must have adequate liver function (bilirubin _< 2.0 mg/dl, SGOT less than
1.5 times normal (unless it is due to disease), adequate renal function (creatinine
<_ 1.5 mg/dl, creatinine clearance >_ 60 ml/min/1.73 m2) and normal electrolytes.
- Patients should have a granulocyte count > 500/uL and a platelet count >_ 100,000/uL
(unless due to disease involvement of the bone marrow).
- Male and female patients of child-bearing age should use effective methods of
contraception, if sexually active.
- Patients with active infections or significant medical conditions other than their
disease (LCH) shall be excluded.