Tier 2 treatment options
| Drug . | Dose . | Response rate (%)* . | Time to response . | Selected toxicities . |
|---|---|---|---|---|
| 6-mercaptopurine | 50-75 mg/m2 orally once per day | 83 | Not reported | Hepatotoxicity, neutropenia, infection, pancreatitis |
| Azathioprine | 1-2 mg/kg orally once per day (maximum 150 mg/d) | 40-60 | 3-6 mo | Hepatotoxicity, neutropenia, infection, pancreatitis |
| Cyclosporin A | 5-6 mg/kg/d orally divided into 2 doses per day (titrate to blood levels of 100-200 ng/mL) | 30-60 | 3-4 wk | Nephrotoxicity, hypertension, tremor, parathesias, gingival hyperplasia |
| Cyclophosphamide | 0.3-1.0 g/m2 IV repeated once every 2 to 4 weeks × 1 to 3 doses; 50-200 mg orally once per day; after response has been achieved, dose can be tapered to 50 mg | 24-85 | 1-16 wk | Neutropenia, nausea/vomiting, infertility, secondary malignancy |
| Danazol | 50-800 mg/d orally divided into 2 to 4 doses per day | 10-70 | 3-6 mo | Hepatotoxicity, virilization, amenorrhea |
| Dapsone | 75-100 mg orally once per day | 40-75 | 3 wk | Hemolysis (in patients with G6PD deficiency), rash, nausea, methemoglobinuria |
| Mycophenolate mofetil | 250-1000 mg orally twice per day | 11-80 | 4-6 wk | Headache, diarrhea, nausea, anorexia, infection |
| Vinca alkaloids | Vincristine: 1 to 2 mg IV once per week × 3 weeks Vinblastine: 10 mg IV once per week × 3 weeks | 10-75 | 5-7 d | Peripheral neuropathy, vesication at infusion site, constipation, fever, neutropenia |
| Drug . | Dose . | Response rate (%)* . | Time to response . | Selected toxicities . |
|---|---|---|---|---|
| 6-mercaptopurine | 50-75 mg/m2 orally once per day | 83 | Not reported | Hepatotoxicity, neutropenia, infection, pancreatitis |
| Azathioprine | 1-2 mg/kg orally once per day (maximum 150 mg/d) | 40-60 | 3-6 mo | Hepatotoxicity, neutropenia, infection, pancreatitis |
| Cyclosporin A | 5-6 mg/kg/d orally divided into 2 doses per day (titrate to blood levels of 100-200 ng/mL) | 30-60 | 3-4 wk | Nephrotoxicity, hypertension, tremor, parathesias, gingival hyperplasia |
| Cyclophosphamide | 0.3-1.0 g/m2 IV repeated once every 2 to 4 weeks × 1 to 3 doses; 50-200 mg orally once per day; after response has been achieved, dose can be tapered to 50 mg | 24-85 | 1-16 wk | Neutropenia, nausea/vomiting, infertility, secondary malignancy |
| Danazol | 50-800 mg/d orally divided into 2 to 4 doses per day | 10-70 | 3-6 mo | Hepatotoxicity, virilization, amenorrhea |
| Dapsone | 75-100 mg orally once per day | 40-75 | 3 wk | Hemolysis (in patients with G6PD deficiency), rash, nausea, methemoglobinuria |
| Mycophenolate mofetil | 250-1000 mg orally twice per day | 11-80 | 4-6 wk | Headache, diarrhea, nausea, anorexia, infection |
| Vinca alkaloids | Vincristine: 1 to 2 mg IV once per week × 3 weeks Vinblastine: 10 mg IV once per week × 3 weeks | 10-75 | 5-7 d | Peripheral neuropathy, vesication at infusion site, constipation, fever, neutropenia |
G6PD, glucose 6-phosphate dehydrogenase.
Studies vary in their definition of response.