Cytotoxic chemotherapy and allogeneic SCT

The acute and lymphoma types of ATL respond poorly to combination cytotoxic chemotherapy and allogeneic SCT, with a median OS under 1 year.[19-21] Less than 10% of patients who received combination therapy were alive after 4 years in one retrospective study.[21] Durable remissions have been reported after allogeneic SCT and even after subsequent donor lymphocyte infusion for relapses after transplant.[22-24][Level of evidence C3] Among 815 patients who underwent allogeneic SCT in two retrospective reviews, the 3-year OS rates were 36% and 26%.[25,26][Level of evidence C1]

The cytotoxic chemotherapy backbone for patients with ATL in the United States and Europe is the CHOP or CHO(E)P regimen (cyclophosphamide, doxorubicin, vincristine, etoposide, and prednisone). Brentuximab vedotin has been added to CHOP in rare cases when patients express CD30. For the acute and lymphoma versions of ATL, a series of clinical trials in Japan established VCAP-AMP-VECP as the standard first-line treatment.[2,27,28] This regimen has generally not been implemented outside of Asia since a trial comparing VCAP-AMP-VECP versus CHOP-14 did not show statistically improved OS (3-year OS rate, 24% vs. 13%; P = nonsignificant).

Allogeneic SCT is frequently used for patients achieving first or later remission, although results are largely anecdotal.[29,30]