Treatment Options for Recurrent or Refractory Lymphoblastic Lymphoma

Nelarabine or nelarabine-containing chemotherapy regimens (nelarabine, cyclophosphamide, and etoposide).[32-35]

ICE regimen (ifosfamide, carboplatin, and etoposide).[36]

Bortezomib with block 1 (four-drug induction), block 2 (cyclophosphamide, etoposide, and high-dose methotrexate), and block 3 (high-dose cytarabine and PEG-asparaginase).[37]

Daratumumab with chemotherapy.[38]

Allogeneic hematopoietic stem cell transplant (HSCT).[39,40]

A COG phase II study of nelarabine (compound 506U78) as a single agent demonstrated a response rate of 40%.[32]

A phase IV multicenter study of patients with recurrent or refractory T-cell leukemia/lymphoma (n = 28, 11 lymphoma) were treated with single-agent nelarabine.[33]

A phase IV multicenter study of patients with recurrent or refractory T-cell leukemia/lymphoma (n = 28, 11 lymphoma) were treated with single-agent nelarabine.[33]

A complete response rate of 36% was observed.

Three small series have treated patients with recurrent or refractory T-cell leukemia/lymphoma using nelarabine, cyclophosphamide, and etoposide.[34,41,42]

Three small series have treated patients with recurrent or refractory T-cell leukemia/lymphoma using nelarabine, cyclophosphamide, and etoposide.[34,41,42]

One study treated 27 patients.[34]

Three small series have treated patients with recurrent or refractory T-cell leukemia/lymphoma using nelarabine, cyclophosphamide, and etoposide.[34,41,42] › One study treated 27 patients.[34]

A partial/complete response rate of 85% was observed.

Three small series have treated patients with recurrent or refractory T-cell leukemia/lymphoma using nelarabine, cyclophosphamide, and etoposide.[34,41,42] › One study treated 27 patients.[34]

However, 13% of patients developed greater than grade 3 neurotoxicity, and three patients died of neurotoxicity.

Three small series have treated patients with recurrent or refractory T-cell leukemia/lymphoma using nelarabine, cyclophosphamide, and etoposide.[34,41,42] › One study treated 27 patients.[34]

Of the four lymphoma patients, one patient achieved a partial remission, but all patients eventually had disease progression.

Three small series have treated patients with recurrent or refractory T-cell leukemia/lymphoma using nelarabine, cyclophosphamide, and etoposide.[34,41,42]

The second study treated seven patients.[41]

Three small series have treated patients with recurrent or refractory T-cell leukemia/lymphoma using nelarabine, cyclophosphamide, and etoposide.[34,41,42] › The second study treated seven patients.[41]

The partial/complete response rate was 100%.

Three small series have treated patients with recurrent or refractory T-cell leukemia/lymphoma using nelarabine, cyclophosphamide, and etoposide.[34,41,42] › The second study treated seven patients.[41]

Of the two lymphoma patients, both achieved partial responses but later progressed.

Three small series have treated patients with recurrent or refractory T-cell leukemia/lymphoma using nelarabine, cyclophosphamide, and etoposide.[34,41,42]

The third study treated ten patients.[42]

Three small series have treated patients with recurrent or refractory T-cell leukemia/lymphoma using nelarabine, cyclophosphamide, and etoposide.[34,41,42] › The third study treated ten patients.[42]

Forty-four percent of the patients had any response (complete response, complete response with incomplete platelet recovery, or partial response), and the complete response rate was 33%.

Three small series have treated patients with recurrent or refractory T-cell leukemia/lymphoma using nelarabine, cyclophosphamide, and etoposide.[34,41,42] › The third study treated ten patients.[42]

Two of ten patients had severe peripheral neuropathy.

On the AALL07P1 (NCT00873093) trial, ten patients with T-cell lymphoblastic lymphoma in first relapse were treated with bortezomib added to a four-drug induction regimen.[37]

On the AALL07P1 (NCT00873093) trial, ten patients with T-cell lymphoblastic lymphoma in first relapse were treated with bortezomib added to a four-drug induction regimen.[37]

Seven patients had a response; one patient had a complete response, two patients had unconfirmed complete responses, and four patients had partial responses.

An international collaboration studied the use of daratumumab plus two cycles of chemotherapy in ten patients with relapsed or refractory T-cell lymphoblastic lymphoma.[38]

An international collaboration studied the use of daratumumab plus two cycles of chemotherapy in ten patients with relapsed or refractory T-cell lymphoblastic lymphoma.[38]

The overall response rate was 50%, with a 40% complete response rate.

An international collaboration studied the use of daratumumab plus two cycles of chemotherapy in ten patients with relapsed or refractory T-cell lymphoblastic lymphoma.[38]

The addition of daratumumab did not significantly increase the toxicity of the chemotherapy, but most patients experienced infusion-related toxicity (70%; grades 1 and 2).

A BFM study showed an OS rate of 14% for patients relapsing after BFM front-line therapy. All patients who survived had undergone an allogeneic HSCT.[31]

A Center for International Blood and Marrow Transplant Research analysis demonstrated that the EFS rate was significantly worse when an autologous (4%) versus allogeneic (40%) donor stem cell source was used. All treatment failures resulted from progressive disease.[39]