Multicenter analysis of treatment outcomes in adult patients with lymphoblastic lymphoma who received hyper-CVAD induction followed by hematopoietic stem cell transplantation

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Abstract

The hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone (hyper-CVAD) regimen has been widely used for lymphoblastic lymphoma (LBL) as a primary treatment. However, there is few data about its treatment outcome in Asian patients. Thus, we conducted this study to evaluate the efficacy of hyper-CVAD induction and stem cell transplantation (SCT) consolidation in LBL patients. The treatment responses of 49 patients treated with the hyper-CVAD regimen were retrospectively analyzed in 13 institutions. Given 24 patients who responded to hyper-CVAD underwent consolidation treatment with SCT, overall survival (OS) and progression-free survival (PFS) of patients who received SCT were compared with patients who did not. The overall response rate was 79 %: 73 % (36/49) complete responses, 6 % (3/49) partial responses, and 4 % (2/49) induction deaths. The major limitation for the delivery of the planned hyper-CVAD cycles was hematological toxicity. Among 39 responders, 24 patients underwent autologous (n = 16) and allogeneic SCT (n = 8) consolidation. Their 3-year OS and PFS rates were 76 and 78 %, respectively, and there was no difference in survival outcomes between autologous and allogeneic SCT. However, 15 patients without SCT consolidation showed poorer PFS even though they all achieved complete response. Thus, only seven patients maintained their response at the time of analysis. In conclusion, the hyper-CVAD regimen is effective for remission induction in LBL, and SCT consolidation after hyper-CVAD induction produced better clinical outcomes than did continuation of hyper-CVAD.

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Outcome with the hyper-CVAD regimens in lymphoblastic lymphoma.

Deborah A Thomas, Susan O'Brien, Jorge Cerna Cortes … (2004)

Therapy of lymphoblastic lymphoma (LL) has evolved with use of chemotherapy regimens modeled after those for acute lymphocytic leukemia (ALL). We treated 33 patients with LL with the intensive chemotherapy regimens hyper-CVAD (fractionated cyclophosphamide, vincristine, Adriamycin, and dexamethasone) or modified hyper-CVAD used for ALL at our institution. Induction consolidation was administered with 8 or 9 alternating cycles of chemotherapy over 5 to 6 months with intrathecal chemotherapy prophylaxis, followed by maintenance therapy. Consolidative radiation therapy was given to patients with mediastinal disease at presentation. No consolidation with autologous or allogeneic stem cell transplantation was performed. At diagnosis, 80% were T-cell immunophenotype, 70% were stages III to IV, 70% had mediastinal involvement, and 9% had central nervous system (CNS) disease. Of the patients, 30 (91%) achieved complete remission, and 3 (9%) achieved partial response. Within a median of 13 months, 10 patients (30%) relapsed or progressed. Estimates for 3-year progression-free and overall survival for the 33 patients were 66% and 70%, respectively. Estimates for the patients with known T-cell immunophenotype were 62% and 67%, respectively. No parameters (eg, age, stage, serum lactate dehydrogenase [LDH], beta(2) microglobulin) appeared to influence outcome except for CNS disease at presentation. Modification of the hyper-CVAD regimen with anthracycline intensification did not improve outcome. Other modifications of the program could include incorporation of monoclonal antibodies and/or nucleoside analogs, particularly for slow responders or those with residual mediastinal disease.

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Lymphoblastic lymphoma.

Dieter Hoelzer, Maurilio Ponzoni, Sergio Cortelazzo … (2011)

Lymphoblastic lymphoma (LBL) is a neoplasm of immature B cells committed to the B-(B-LBL) or T-cell lineage (T-LBL) that accounts for approximately 2% of all lymphomas. From a histopathological point of view, blasts may be encountered in tissue biopsy and/or bone marrow (BM). In tissue sections, LBL is generally characterized by a diffuse or, as in lymph nodes and less commonly, paracortical pattern. Although histological features are usually sufficient to distinguish lymphoblastic from mature B- or T-cell neoplasms, a differential diagnosis with blastoid variant of mantle cell lymphoma, Burkitt lymphoma or myeloid leukemia may arise in some cases. Of greater importance is the characterization of immunophenotype by flow cytometry. In B-LBL, tumour cells are virtually always positive for B cell markers CD19, CD79a and CD22. They are positive for CD10, CD 24, PAX5, and TdT in most cases, while the expression of CD20 and the lineage independent stem cell antigen CD34 is variable and CD45 may be absent. Surface immunoglobulin is usually absent. In T-LBL, neoplastic cells are usually TdT positive and variably express CD1a, CD2, CD3, CD4, CD5, CD7 and CD8. The only reliable lineage-specific is surface CD3. Most B-LBL have clonal rearrangements of the Ig heavy chain or less frequently of light chain genes. T-cell receptor γ or β chain gene rearrangements may be seen in a significant number of cases, but rearrangements are not helpful for lineage assignment. LBL occurs more commonly in children than in adults, mostly in males. Although 80% of precursor B-cell neoplasms present as acute leukemias, with BM and peripheral blood (PB) involvement, a small proportion present with a mass lesion and have <25% blasts in the BM. Unlike precursor T-LBL, mediastinal masses and involvement of BM are rare, but lymph nodes and extranodal sites are more frequently involved. T-LBL patients, compared to those with B-LBL, show younger age, a higher rate of mediastinal tumours or BM involvement. Patients are usually males in their teens to twenties and present with lymphadenopathy in cervical, supraclavicular and axillary regions, or with a mediastinal mass. In most patients the mediastinal mass is anterior, bulky, and associated with pleural effusions, superior vena cava syndrome, tracheal obstruction, and pericardial effusions. They frequently present with advanced disease, B symptoms and elevated serum LDH levels. Abdominal involvement (liver and spleen) is unusual. LBL is highly aggressive, but frequently curable with current therapy. The prognosis in all age groups has dramatically improved with the use of intensive ALL-type chemotherapy regimes, with a disease-free survival of 73-90% in children and 45-72% in adults. Intensive intrathecal chemotherapy prophylaxis is required to reduce the CNS relapse incidence, while the role of prophylactic cranial irradiation is unclear. Consolidation mediastinal irradiation may decrease mediastinal relapse. Patients with adverse prognostic features should be considered for high-dose chemotherapy and SCT. Autologous SCT has been shown to produce similar good results as chemotherapy alone, and allogeneic SCT is likely to be a more appropriate option for patients who are beyond first remission or with more advanced disease. Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.

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Outcome of adult patients with T-lymphoblastic lymphoma treated according to protocols for acute lymphoblastic leukemia.

Dieter Hoelzer, Nicola Gökbuget, Werner Digel … (2002)

We treated 45 adult patients with T-lymphoblastic lymphoma (T-LBL) (age range 15-61 years) with 2 protocols designed for adult acute lymphoblastic leukemia (ALL). An encouraging cure rate of 90% was recently reported for T-LBL in children treated with a similar approach. In our study, an 8-drug standard induction was administered over 8 weeks including prophylactic cranial (24 Gy) and mediastinal irradiation (24 Gy) followed by consolidation and reinduction therapy. At diagnosis, 91% of the 45 patients showed a mediastinal tumor and 40% had pleural/pericardial effusions; 73% of the patients had stage III/IV disease. Overall, 42 patients (93%) achieved a complete remission (CR), 2 patients (4%) achieved a partial remission, and 1 patient (2%) died during induction. In patients with stage I-III disease (n = 18) the CR rate was 100% compared with 89% in stage IV (n = 27). There were 15 patients who relapsed (36%) within 12 months. The majority of relapses (47%) occurred in the mediastinum (n = 7) despite mediastinal irradiation with 24 Gy in 6 out of 7 patients. The estimates for overall survival, continuous CR, and disease-free survival at 7 years are 51%, 65%, and 62%, respectively. Stage, age, lactate dehydrogenase, and all other parameters had no influence on achievement of CR or outcome. This study demonstrates in a large cohort of patients with adult T-LBL that a high CR rate and a favorable outcome can be achieved with an ALL-type regimen. Mediastinal recurrence was the major obstacle and further improvement by intensification of chemotherapy, increased dose of mediastinal irradiation (36 Gy), and extended indications for stem cell transplantation seem to be required.