| Testing for IFN sensitivity may help decide between IFN-α and allogeneic SCT. Testing for IFN-α sensitivity may require some time, but treatment of low-risk patients is never urgent. In the table, the term investigational treatment encompasses a number of procedures (e.g. intensified chemotherapy with autologous stem cell rescue, allogeneic SCT from mismatched donors or in the elderly) and novel agents. However, the first-line investigational option to be considered should be the BCR-ABL tyrosine-kinase inhibitor STI571, either alone or in combination. |
Age < 30 years, any risk
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Age 30-55 years, low risk
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Test for IFN-α sensitivity. If test is positive, first-line option is IFN-α. The surrogate target for long survival is achieving a major cytogenetic response in 1 year and a complete cytogenetic response in 2 years. If test is negative, first-line option is allogeneic SCT from an HLA-identical sibling or an HLA-matched unrelated donor. If a suitable donor is not available, investigational treatment.
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Age 30-55 years, non-low risk
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First-line option is allogeneic SCT from an HLA-identical sibling or an HLA-matched unrelated donor. If a suitable donor is not available, investigational treatment.
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Age > 55 years, low risk
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Test for IFN-α sensitivity. If test is positive, first-line option is IFN-α as for younger patients. If test is negative or IFN-α is not tolerated, investigational treatment.
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Age > 55, non-low risk
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First-line treatment is investigational. If investigational treatment is not feasible or not tolerated, second line is hydroxyurea.
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