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. 2017 Jun 8;57(3):87–97. doi: 10.3960/jslrt.17008

Fig. 2.

Fig. 2

Treatment algorithm for ATL patients.

The multi-agent chemotherapies are recommended for patients with aggressive ATL as first-line treatment, such as VCAP-AMP-VECP and CHOP. If these patients respond to first-line chemotherapy and have a HLA-matched donor, subsequent allo-HSCT is recommended. Mogamulizumab is used for patients whose ATL cells express CC chemokine receptor 4. Patients with refractory or relapsed aggressive ATL are treated with multi-agent chemotherapy containing drugs not used in the prior regimen or single-agent therapies. The combination of gemcitabine and oxaliplatin, pralatrexate, or DHAP were used as investigator’s choice regimens in a prospective study. The combination of interferon-α and zidovudine (IFN-AZT) have been used for the treatment of acute, chronic, and smoldering types outside Japan. The retrospective study showed that low-dose IFN-AZT with chemotherapy is also an option for lymphoma type. Regarding indolent ATL, watchful waiting including skin-directed therapy for skin lesions or IFN-AZT are recommended. Chemotherapy is applied if transformation to an aggressive type occurs.

Abbreviations: allo-HSCT, allogeneic-hematopoietic stem cell transplantation; CHOP, consisting of cyclophosphamide, doxorubicin, vincristine, and prednisolone; VCAP-AMP-VECP, sequential combination chemotherapy consisting of VCAP (vincristine, cyclophosphamide, doxorubicin, and prednisolone), AMP (doxorubicin, ranimustine, and prednisolone), and VECP (vindesine, etoposide, carboplatin, and prednisolone); ATL-GCSG, consisting of vincristine, vindesine, doxorubicin, mitoxantrone, cyclophosphamide, etoposide, ranimustine, and prednisolone with prophylactic support by granulocyte colony-stimulating factor; Modified EPOCH consisting of etoposide, prednisolone, vincristine, carboplatin, and doxorubicin: cyclophosphamide used in original EPOCH was substituted for carboplatin.; DHAP, consisting of dexamethasone, high-dose cytarabine, and cisplatin; Hyper-CVAD, consisting of cyclophosphamide, doxorubicin, vincristine, and dexamethasone.