Treatment of HCL
| Initial treatment |
| Cladribine administered as continuous intravenous infusion per day, days 1 through 7,55,80 intravenously over 2 hours once per day for 5 days,56,79 intravenously over 2 hours once per week for 5 or 6 weeks,55 or subcutaneously once per day for 5 days.22,23,55 |
| Pentostatin administered intravenously once every 2 weeks to patients with attention to renal function.48,49 Lower doses have been used under special circumstances. |
| Treatment at relapse |
| Confirmation of the initial diagnosis is important, including review of data to determine whether previous therapy was correct and whether poor-risk features were identified (eg, severe anemia, spleen >10 cm below the left costal margin, abnormal immunophenotypic profile, absence of BRAFV600E mutation). |
| Determination of the indication for re-treatment equivalent to the initial criteria, including symptomatic disease (eg, splenomegaly) or progressive anemia, thrombocytopenia, or neutropenia. |
| If previous remission was >24 months, then consider re-treatment with a purine analog possibly combined with an anti-CD20 monoclonal antibody, or a clinical trial. |
| If previous remission was >60 months, consider re-treatment with initial therapy. |
| If previous remission was <24 months, consider alternative therapy, including investigational agents, after confirming accuracy of diagnosis. |
| Older therapeutic approaches may still offer benefit (eg, interferon alpha splenectomy, rituximab). |
| Initial treatment |
| Cladribine administered as continuous intravenous infusion per day, days 1 through 7,55,80 intravenously over 2 hours once per day for 5 days,56,79 intravenously over 2 hours once per week for 5 or 6 weeks,55 or subcutaneously once per day for 5 days.22,23,55 |
| Pentostatin administered intravenously once every 2 weeks to patients with attention to renal function.48,49 Lower doses have been used under special circumstances. |
| Treatment at relapse |
| Confirmation of the initial diagnosis is important, including review of data to determine whether previous therapy was correct and whether poor-risk features were identified (eg, severe anemia, spleen >10 cm below the left costal margin, abnormal immunophenotypic profile, absence of BRAFV600E mutation). |
| Determination of the indication for re-treatment equivalent to the initial criteria, including symptomatic disease (eg, splenomegaly) or progressive anemia, thrombocytopenia, or neutropenia. |
| If previous remission was >24 months, then consider re-treatment with a purine analog possibly combined with an anti-CD20 monoclonal antibody, or a clinical trial. |
| If previous remission was >60 months, consider re-treatment with initial therapy. |
| If previous remission was <24 months, consider alternative therapy, including investigational agents, after confirming accuracy of diagnosis. |
| Older therapeutic approaches may still offer benefit (eg, interferon alpha splenectomy, rituximab). |