Outcome with the hyper-CVAD regimens in lymphoblastic lymphoma

Abstract

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], ␤ 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

Absolute lymphocyte count is a novel prognostic indicator in ALL and AML

“…A subset of our young adult ALL patients (n 5 25) received hyper-CVAD therapy (dexamethasone, vincristine, doxorubicin, cyclophosphamide, then methotrexate, cytarabine, 38 cycles). 18,19 The use of hyper-CVAD is not a standard therapy in any of the major pediatric oncology groups; however, it is standard therapy given by the adult leukemia service at our institution. Induction therapy for AML consisted of anthracycline (daunorubicin or idarubicin) and cytarabine with or without etoposide.…”

Absence of terminal deoxynucleotidyl transferase expression identifies a subset of high-risk adult T-lymphoblastic leukemia/lymphoma

“…Complete remission can be achieved initially in over 90% of T-ALL/LBL patients, but a substantial subset of patients eventually develop relapsed disease. The 5-year overall survival for patients with T-ALL/LBL is over 80% in children 1 and B50% in adults, [2][3][4][5] with most relapses occurring within 2 years after initial remission. 4 Patients with relapsed T-ALL/LBL usually respond poorly to augmented salvage chemotherapy and allogeneic hematopoietic stem cell transplant.…”

Allogeneic transplantation is not superior to chemotherapy in most patients over 40 years of age with Philadelphia‐negative acute lymphoblastic leukemia in first remission

“…Patients who achieved CR within 70 days from initiation of induction therapy were included in the analysis; this extended time was used in order to address a limitation of retrospectively analyzing patients who may be induced at other institutions and had late remission assessments. This cutoff is also in line with many protocols that do not declare that a patient has failed to achieve remission before completing reinduction [17], consolidation [18][19][20] or treating with an escalated, risk adapted intensification [21].…”

“…1 and 2; P value nonsignificant for both comparisons). The 3-year CIR and NRM were 61% [41-76] and 9% [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21] for the chemotherapy-only group and 28% and 32% for the transplant group (Table II; Fig. 3; P 5 0.011 for CIR and P 5 0.014 for NRM).…”