Treatment Options for Anaplastic Large Cell Lymphoma

A prospective randomized trial included 452 patients with CD30-positive T-cell lymphoma (CD30 expression >10%). Of these patients, 70% had ALCL (22% with ALK-positive disease and 48% with ALK-negative disease). The trial compared the previously used standard regimen, CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone), with brentuximab vedotin (an anti-CD30 monoclonal antibody conjugated to a cytotoxic agent) combined with cyclophosphamide, doxorubicin, and prednisone (A+CHP regimen).[6]

A prospective randomized trial included 452 patients with CD30-positive T-cell lymphoma (CD30 expression >10%). Of these patients, 70% had ALCL (22% with ALK-positive disease and 48% with ALK-negative disease). The trial compared the previously used standard regimen, CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone), with brentuximab vedotin (an anti-CD30 monoclonal antibody conjugated to a cytotoxic agent) combined with cyclophosphamide, doxorubicin, and prednisone (A+CHP regimen).[6]

With a median follow-up of 47.6 months, the 5-year overall survival (OS) rates were 70.1% (95% confidence interval [CI], 63.3%–75.9%) for patients who received A+CHP and 61.0% (95% CI, 54.0%–67.3%) for patients who received CHOP (hazard ratio [HR], 0.72; 95% CI, 0.53–0.99).[7][Level of evidence A1]

A prospective randomized trial included 452 patients with CD30-positive T-cell lymphoma (CD30 expression >10%). Of these patients, 70% had ALCL (22% with ALK-positive disease and 48% with ALK-negative disease). The trial compared the previously used standard regimen, CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone), with brentuximab vedotin (an anti-CD30 monoclonal antibody conjugated to a cytotoxic agent) combined with cyclophosphamide, doxorubicin, and prednisone (A+CHP regimen).[6]

The 5-year progression-free survival (PFS) rates were 51.4% (95% CI, 42.8%–59.4%) for patients who received A+CHP and 43.0% (95% CI, 35.8%–50.0%) for patients who received CHOP (HR, 0.70; 95% CI, 0.53–0.91).

A prospective randomized trial included 452 patients with CD30-positive T-cell lymphoma (CD30 expression >10%). Of these patients, 70% had ALCL (22% with ALK-positive disease and 48% with ALK-negative disease). The trial compared the previously used standard regimen, CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone), with brentuximab vedotin (an anti-CD30 monoclonal antibody conjugated to a cytotoxic agent) combined with cyclophosphamide, doxorubicin, and prednisone (A+CHP regimen).[6]

This established A+CHP as a new option for patients with ALCL or other CD30-positive T-cell lymphomas, such as angioimmunoblastic T-cell lymphoma and peripheral T-cell lymphoma, not otherwise specified.

For patients with relapsed disease, anecdotal responses have been reported for brentuximab vedotin,[8-11] belinostat,[12] romidepsin,[13] and pralatrexate.[14][Level of evidence C3]

In a phase II study (NCT00866047), 66% of 58 patients attained a complete response with brentuximab vedotin.[11]

In a phase II study (NCT00866047), 66% of 58 patients attained a complete response with brentuximab vedotin.[11]

At a median follow-up of 58 months, the 5-year PFS rate was 57% (95% CI, 41%–74%), and the 5-year OS rate was 79% (95% CI, 65%–92%). Of the patients achieving a complete response, 42% underwent hematopoietic stem cell transplant (SCT).[11][Level of evidence C3]

In a retrospective review, 39 patients with relapsed disease had a 3-year PFS rate of 50% after autologous or allogeneic SCT.[15][Level of evidence C2]

A retrospective review of 84 patients with ALK-negative ALCL suggested a survival benefit with autologous SCT. This hypothesis requires confirmation in a randomized prospective trial.[16][Level of evidence C3]

A retrospective study included 182 patients with relapsed or refractory ALCL (23% ALK-positive, 21% ALK-negative, and 56% ALK-unknown) who underwent allogeneic SCT.[17]

A retrospective study included 182 patients with relapsed or refractory ALCL (23% ALK-positive, 21% ALK-negative, and 56% ALK-unknown) who underwent allogeneic SCT.[17]

The 5-year PFS rate was 41% (95% CI, 34%–49%), and the 5-year OS rate was 41% (95% CI, 49%–64%).[17][Level of evidence C3]

A retrospective study included 182 patients with relapsed or refractory ALCL (23% ALK-positive, 21% ALK-negative, and 56% ALK-unknown) who underwent allogeneic SCT.[17]

On multivariate analysis, African American race (HR, 2.7; 95% CI, 1.6–4.8; P < .001) and refractory disease at time of allogeneic SCT (HR, 3.2; 95% CI, 1.6–6.2; P < .001) were predictive factors for inferior OS.

A retrospective study included 182 patients with relapsed or refractory ALCL (23% ALK-positive, 21% ALK-negative, and 56% ALK-unknown) who underwent allogeneic SCT.[17]

Despite ALK positivity being a favorable prognostic factor, outcomes after allogeneic SCT in this study did not vary significantly according to ALK status.