Table 2.

Treatment strategy for adult T-cell leukemia/lymphoma (ATLL).

1. Indolent-type ATLL: Smoldering- or favorable chronic-type

(1) Watchful waiting for asymptomatic patients

(2) Interferon-α (IFN-α)/zidovudine (AZT) or watchful waiting for symptomatic patients

(3) Skin lesion:

Local therapy; Topical steroids, Ultraviolet light, Radiation

Systemic therapy; Steroids, Oral retinoids, Single agent chemotherapy

2. Aggressive-type ATLL: Unfavorable chronic-, lymphoma- or acute-type

(1) Chemotherapy:

VCAP-AMP-VECP

CHOP or less-toxic regimen for elderly patients

(2) VCAP-AMP-VECP + mogamulizumab

(3) Allogeneic hematopoetic stem cell transplantation (allo-HSCT)

(4) IFN-α/AZT (except for lymphoma-type)

3. Relapse or refractory ATLL

(1) Mogamulizumab

(2) Allo-HSCT

(3) New agents under clinical trial:

Brentuximab vedotin, Bortezomib, Lenalidomide, Panobinostat, Forodesine

Pralatrexate, Denileukin diftitox

(4) Vaccine (autologous dendritic cells with tax-peptide)

VCAP-AMP-VECP: vincristine, cyclophosphamide, doxorubicin, and prednisolone (VCAP); doxorubicin, ranimustine, and prednisolone (AMP); and vindesine, etoposide, carboplatin, and prednisolone (VECP). CHOP: doxorubicin, cyclophosphamide, vincristine and prednisone.