scholarly journals Successful therapy of convoluted T-lymphoblastic lymphoma in the adult

Blood ◽  
1983 ◽  
Vol 61 (1) ◽  
pp. 92-98 ◽  
Author(s):  
AM Levine ◽  
SJ Forman ◽  
PR Meyer ◽  
SC Koehler ◽  
H Liebman ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Median Survival ◽  
Bone Marrow Involvement ◽  
Cranial Irradiation ◽  
Lymphoblastic Lymphoma ◽  
Intrathecal Methotrexate ◽  
Induction Phase ◽  
Relapse Free Survival ◽  
Free Survival ◽  
T Lymphoblastic Lymphoma

Abstract Fifteen adult patients with biopsy-proven convoluted T-lymphoblastic lymphoma were treated with an aggressive regimen, modified from the LSA2-L2 protocol used for childhood lymphoma. The treatment schema consisted of induction phase, including cyclophosphamide, vincristine, prednisone, adriamycin, and 2000 rads to mediastinum, as well as intrathecal methotrexate. Consolidation phase included cytosine arabinoside, 6-thioguanine, L-asparaginase, and CCNU, along with cranial irradiation and further intrathecal methotrexate. Maintenance consisted of cyclical chemotherapy and intrathecal methotrexate, continuing for a total of 3 yr. Median age in the group was 25 yr (range 16–73). There were 8 males and 7 females. At diagnosis, 9 patients had mediastinal involvement, and 9 had bone marrow involvement. Five of these demonstrated malignant cells in the peripheral blood. Complete clinical response was attained in 11 patients. Three patients achieved partial response. Four complete responders have relapsed, 1 in the central nervous system at 6 mo. and 1 in nodal sites at 3 mo, 1 in multiple sites at 24 mo. and 1 in bone marrow at 42 mo while off all chemotherapy for 6 mos. At this time, median survival of all patients is 28.3 mo. and median relapse-free survival is 21 mo. The median survival for complete responders in excess of 71 mo. while the median relapse-free survival for this group is 41 mo.

Successful therapy of convoluted T-lymphoblastic lymphoma in the adult

Blood ◽  
1983 ◽  
Vol 61 (1) ◽  
pp. 92-98
Author(s):  
AM Levine ◽  
SJ Forman ◽  
PR Meyer ◽  
SC Koehler ◽  
H Liebman ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Median Survival ◽  
Bone Marrow Involvement ◽  
Cranial Irradiation ◽  
Lymphoblastic Lymphoma ◽  
Intrathecal Methotrexate ◽  
Induction Phase ◽  
Relapse Free Survival ◽  
Free Survival ◽  
T Lymphoblastic Lymphoma

Fifteen adult patients with biopsy-proven convoluted T-lymphoblastic lymphoma were treated with an aggressive regimen, modified from the LSA2-L2 protocol used for childhood lymphoma. The treatment schema consisted of induction phase, including cyclophosphamide, vincristine, prednisone, adriamycin, and 2000 rads to mediastinum, as well as intrathecal methotrexate. Consolidation phase included cytosine arabinoside, 6-thioguanine, L-asparaginase, and CCNU, along with cranial irradiation and further intrathecal methotrexate. Maintenance consisted of cyclical chemotherapy and intrathecal methotrexate, continuing for a total of 3 yr. Median age in the group was 25 yr (range 16–73). There were 8 males and 7 females. At diagnosis, 9 patients had mediastinal involvement, and 9 had bone marrow involvement. Five of these demonstrated malignant cells in the peripheral blood. Complete clinical response was attained in 11 patients. Three patients achieved partial response. Four complete responders have relapsed, 1 in the central nervous system at 6 mo. and 1 in nodal sites at 3 mo, 1 in multiple sites at 24 mo. and 1 in bone marrow at 42 mo while off all chemotherapy for 6 mos. At this time, median survival of all patients is 28.3 mo. and median relapse-free survival is 21 mo. The median survival for complete responders in excess of 71 mo. while the median relapse-free survival for this group is 41 mo.

scholarly journals Comparison of central nervous system prophylaxis with cranial radiation and intrathecal methotrexate versus intrathecal methotrexate alone in acute lymphoblastic leukemia

Blood ◽  
1983 ◽  
Vol 62 (2) ◽  
pp. 241-250
Author(s):  
FS Muriel ◽  
E Svarch ◽  
S Pavlovsky ◽  
M Eppinger-Helft ◽  
J Braier ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Acute Lymphoblastic Leukemia ◽  
Lymphoblastic Leukemia ◽  
Cranial Irradiation ◽  
Intrathecal Methotrexate ◽  
Relapse Free Survival ◽  
Free Survival ◽  
Cns Relapse ◽  
Wbc Count

In acute lymphoblastic leukemia (ALL), central nervous system (CNS) prophylaxis with cranial irradiation plus 5 doses of intrathecal methotrexate (i.t. MTX) reduces the incidence of CNS relapse to 7%-15%. However, increased evidence of CNS delayed toxicity started to be recognized as CT scan abnormalities and neuropsychologic alterations, mainly in children. Two questions were analyzed in the present report: (1) Will further doses of i.t. methotrexate and dexamethasone (i.t. MTX- DMT) decrease the incidence of CNS relapse in patients treated early in remission with cranium irradiation plus i.t. MTX-DMT even more? (2) Is i.t. MTX-DMT given during induction and maintenance equally as effective as cranium irradiation plus i.t. MTX-DMT? A randomized study was designed to answer the first question. Incidence of primary CNS relapse in i.t. MTX-DMT-treated patients with a WBC count less than 50,000 was 11% (15 of 135 patients) and was 11% (17 of 150) in the untreated group. In patients with a WBC count greater than 50,000, it was 16% (6/37) in the treated group and 19% (6/31) in the control group. No difference was observed according to treatment in both prognostic groups. Patients in this study were retrospectively compared with a consecutive protocol in which patients received 3 doses of i.t. MTX-DMT alone during induction plus 3 doses weekly during the first month of remission and every 3 mo thereafter. The incidence of primary CNS leukemia at 60 mo in patients with a WBC count less than 50,000 was 20% in the irradiated group and 32% in the group with i.t. MTX-DMT alone. This difference was not significant. However, the relapse-free survival at 60 mo was 26% and 41%, respectively, (p less than 0.0005). The incidence of primary CNS relapse in patients with a WBC count more than 50,000 at 48 mo was 28% in the irradiated group and 42% in the nonirradiated group. The difference was not significant. The duration of complete remission was similar, remaining at 15% and 16% of patients disease-free at 48 mo, respectively. We conclude that (A) after cranial irradiation plus i.t. MTX-DMT X 5, the use of additional doses of i.t. MTX-DMT is not of further benefit in preventing CNS relapse; (B) the use of i.t. MTX-DMT alone compares similarly with cranial irradiation plus i.t. MTX-DMT in the incidence of CNS relapse; and (C) relapse-free survival and survival in patients with a WBC count less than 50,000 were significantly longer in those without cranial irradiation.

scholarly journals Outcomes of Patients with T-Lymphoblastic Lymphoma Undergoing Allogeneic Stem Cell Transplantation: Retrospective Results from a Single Center

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 5729-5729
Author(s):  
Amandeep Salhotra ◽  
Liana Nikolaenko ◽  
Lu Chen ◽  
Ni-Chun Tsai ◽  
Diane Lynne Smith ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Research Funding ◽  
Chronic Gvhd ◽  
Univariate Analysis ◽  
Bone Marrow Involvement ◽  
Lymphoblastic Lymphoma ◽  
Unrelated Donor ◽  
Remission Status ◽  
T Lymphoblastic Lymphoma

Background: T-lymphoblastic lymphoma (T-LBL) is a highly aggressive non-Hodgkin lymphoma seen in young adults that usually presents with a mediastinal mass and less than 20% bone marrow involvement with neoplastic cells. T-LBL and T-cell ALL are morphologically and immmunophenoytpically similar and are thus frequently grouped together. However there are differences in clinical presentation, cytogenetic features and molecular pathogenesis indicating biologic differences. Patients with relapsed refractory T-LBL have poor prognosis and there is a paucity of data on allogeneic HCT (allo-HCT) outcomes in this setting. Methods: We retrospectively reviewed medical records of 25 consecutive patients with T lymphoblastic lymphoma without prior auto-HCT who underwent allo-HCT at City of Hope from January 2000 to June 2018 after IRB approval was obtained. Descriptive statistics were used to summarize baseline patient demographic, treatment, and disease characteristics. Kaplan-Meier curves and the log-rank test were used to evaluate the overall survival (OS) and progression-free survival (PFS). Cumulative incidences of time to relapse and time to non-relapse mortality (NRM) were calculated with relapse and NRM as competing risks. Cumulative incidences of acute and chronic GVHD were calculated as time to onset of GVHD with relapse and death as competing events for GVHD. Results: 25 patients were included for the analysis. Median age at the time of allo-HCT was 25 years (range 7-70 years). 21 patients (80%) received myeloablative conditioning: FTBI containing regimens (n=20) with FTBI/VP-16 being most commonly used(n=16) and one patient received Bu/Cy based conditioning; 4 patients received reduced intensity conditioning with Flu/Mel. Sibling HCT was performed in 12 patients (48%), while MUD HCT was performed in 13 patients (52%) with fully matched HLA unrelated donor in 9 (36%) and HLA mismatched in 4 (16%) patients. GVHD prophylaxis consisted of tacrolimus/sirolimus (n=10), tacrolimus or cyclosporine/MTX (n=7), tacrolimus/sirolimus/MTX (n=4) and others (n=4) Source of stem cells was PBSC in 19 (76%), bone marrow in 5 (20%), and cord blood in 1 (4%) patients. At the time of allo-HCT, there were a total of 11 (44%) patients in complete remission (CR1 n=4, CR2+ n=7), 9 (36%) patients in partial remission and unknown remission status (n=5). The median follow-up among survivors was 8.9 years (range 2.3-12.1). The 5- and 10-year PFS was 31% (95% CI: 14%-50%). The 5- and 10-year OS was 31% (95% CI: 14%-49%) (Fig.1). Cumulative incidence of relapses at 5 and 10 years were both 36% (95% CI: 18%-55%) while NRM at 5 and 10 years was 33% (95% CI: 15%-52%). At day 100 after allo-HCT, the rates of acute GVHD grade II-IV were 63% (95% CI: 39%-79%) and grade III-IV of 25% (95%CI: 10%-44%). Chronic GVHD rates at 3 years were 60% (95% CI: 37%-77%). In univariate analysis, preHCT remission status did not improve PFS on long term follow up. Conclusions: Our results in patients with T-LBL shows 5-year PFS and OS of 31%, respectively, indicating that a subset of patients can be cured with allo-HCT in the high-risk setting. Disclosures Salhotra: Celgene: Other: Research Support; Kadmon Corporation: Other: Non paid consultant. Popplewell:City of Hope: Employment. Herrera:Kite Pharma: Consultancy, Research Funding; Merck: Consultancy, Research Funding; Genentech, Inc.: Consultancy, Research Funding; Pharmacyclics: Research Funding; AstraZeneca: Research Funding; Immune Design: Research Funding; Bristol-Myers Squibb: Consultancy, Research Funding; Adaptive Biotechnologies: Consultancy; Gilead Sciences: Consultancy, Research Funding; Seattle Genetics: Consultancy, Research Funding. Mei:Seattle Genetics, Inc.: Research Funding. Aldoss:AUTO1: Consultancy; Helocyte: Consultancy, Honoraria, Other: travel/accommodation/expenses; Jazz Pharmaceuticals: Honoraria, Other: travel/accommodation/expenses, Speakers Bureau; Agios: Consultancy, Honoraria. Zain:Seattle Genetics: Honoraria, Speakers Bureau; spectrum: Honoraria.

scholarly journals Comparison of central nervous system prophylaxis with cranial radiation and intrathecal methotrexate versus intrathecal methotrexate alone in acute lymphoblastic leukemia

Blood ◽  
1983 ◽  
Vol 62 (2) ◽  
pp. 241-250 ◽  
Author(s):  
FS Muriel ◽  
E Svarch ◽  
S Pavlovsky ◽  
M Eppinger-Helft ◽  
J Braier ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Acute Lymphoblastic Leukemia ◽  
Lymphoblastic Leukemia ◽  
Cranial Irradiation ◽  
Intrathecal Methotrexate ◽  
Relapse Free Survival ◽  
Free Survival ◽  
Cns Relapse ◽  
Wbc Count

Abstract In acute lymphoblastic leukemia (ALL), central nervous system (CNS) prophylaxis with cranial irradiation plus 5 doses of intrathecal methotrexate (i.t. MTX) reduces the incidence of CNS relapse to 7%-15%. However, increased evidence of CNS delayed toxicity started to be recognized as CT scan abnormalities and neuropsychologic alterations, mainly in children. Two questions were analyzed in the present report: (1) Will further doses of i.t. methotrexate and dexamethasone (i.t. MTX- DMT) decrease the incidence of CNS relapse in patients treated early in remission with cranium irradiation plus i.t. MTX-DMT even more? (2) Is i.t. MTX-DMT given during induction and maintenance equally as effective as cranium irradiation plus i.t. MTX-DMT? A randomized study was designed to answer the first question. Incidence of primary CNS relapse in i.t. MTX-DMT-treated patients with a WBC count less than 50,000 was 11% (15 of 135 patients) and was 11% (17 of 150) in the untreated group. In patients with a WBC count greater than 50,000, it was 16% (6/37) in the treated group and 19% (6/31) in the control group. No difference was observed according to treatment in both prognostic groups. Patients in this study were retrospectively compared with a consecutive protocol in which patients received 3 doses of i.t. MTX-DMT alone during induction plus 3 doses weekly during the first month of remission and every 3 mo thereafter. The incidence of primary CNS leukemia at 60 mo in patients with a WBC count less than 50,000 was 20% in the irradiated group and 32% in the group with i.t. MTX-DMT alone. This difference was not significant. However, the relapse-free survival at 60 mo was 26% and 41%, respectively, (p less than 0.0005). The incidence of primary CNS relapse in patients with a WBC count more than 50,000 at 48 mo was 28% in the irradiated group and 42% in the nonirradiated group. The difference was not significant. The duration of complete remission was similar, remaining at 15% and 16% of patients disease-free at 48 mo, respectively. We conclude that (A) after cranial irradiation plus i.t. MTX-DMT X 5, the use of additional doses of i.t. MTX-DMT is not of further benefit in preventing CNS relapse; (B) the use of i.t. MTX-DMT alone compares similarly with cranial irradiation plus i.t. MTX-DMT in the incidence of CNS relapse; and (C) relapse-free survival and survival in patients with a WBC count less than 50,000 were significantly longer in those without cranial irradiation.

scholarly journals An Unsuspected Finding of t(9;22): A Rare Case of Philadelphia Chromosome-Positive B-Lymphoblastic Lymphoma

2017 ◽  
Vol 2017 ◽  
pp. 1-4 ◽  
Author(s):  
Prajwal Boddu ◽  
C. Cameron Yin ◽  
Rashmi Kanagal-Shamanna ◽  
Guillin Tang ◽  
Beenu Thakral ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Kinase Inhibitors ◽  
Bone Marrow Involvement ◽  
Philadelphia Chromosome ◽  
Lymphoblastic Lymphoma ◽  
Accurate Diagnosis ◽  
Biopsy Specimens ◽  
B Lymphoblastic Lymphoma ◽  
Prophylactic Chemotherapy

While rare, cases of isolated extramedullary disease of B-cell Lymphoblastic Lymphoma (B-LBL) without morphologic bone marrow involvement have been described. In this report, we illustrate the case of an elderly gentleman who presented with isolated testicular and vertebral LBL involvement but had no morphologic bone marrow involvement. The initial plan of treatment was to treat along the lines of Philadelphia negative B-ALL/LBL. During this time, fluorescence in situ hybridization (FISH) and PCR testing for BCR-ABL1 rearrangements were being performed on the marrow specimens as a part of routine diagnostic workup. While the FISH returned negative, PCR testing unexpectedly detected BCR-ABL1 fusion transcripts at a low level of 0.48%. Given their presence, we performed FISH for BCR/ABL1 rearrangement in both testicular and L5 vertebral specimens which were 80–90% positive. He subsequently received rituximab, hyper-CVAD, and dasatinib, along with prophylactic intrathecal prophylactic chemotherapy. The patient achieved a prolonged remission but eventually relapsed, 4 years later. Had it not been for this fortuitous discovery, the patient would not have been treated with tyrosine kinase inhibitors. We emphasize that FISH and PCR testing for BCR-ABL1 rearrangement are integral to arriving at an accurate diagnosis and should be routinely tested on B-LBL biopsy specimens.

CNS involvement in small noncleaved-cell lymphoma: is CNS disease per se a poor prognostic sign?

1991 ◽  
Vol 9 (11) ◽  
pp. 1973-1982 ◽  
Author(s):  
T B Haddy ◽  
M A Adde ◽  
I T Magrath
Keyword(s):  
Bone Marrow ◽  
Cell Lymphoma ◽  
Bone Marrow Involvement ◽  
Serum Lactate Dehydrogenase ◽  
Event Free Survival ◽  
Cns Involvement ◽  
Extensive Disease ◽  
Free Survival ◽  
Cns Disease ◽  
Cns Relapse

Of 120 patients with small noncleaved-cell lymphoma who were entered sequentially on four National Cancer Institute (NCI) protocols, 29 (24%) had CNS involvement at some time in their clinical course. Seventeen had initial CNS involvement, and 12 developed CNS involvement at the time of first relapse. All 29 patients had extensive disease at presentation. The median serum lactate dehydrogenase (LDH) levels at presentation were 1,150 IU/L for patients with initial CNS involvement and 1,083 IU/L for patients with CNS involvement at relapse. CNS disease was significantly associated with serum LDH levels (P less than .0001), bone marrow involvement (P less than .0001), and jaw involvement (P = .018), but not involvement of the abdomen. There were nine long-term survivors among the 29 patients (31%). CNS disease did not appear to confer a worse prognosis on these patients than on patients without CNS involvement who had similar degrees of serum LDH elevation or who had bone marrow involvement, suggesting that extensive disease rather than CNS involvement was responsible for the poor prognosis. Event-free survival for patients with serum LDH levels above 500 IU/L was not different whether CNS disease was present or not (P = .29), nor was event-free survival different for patients with stage IV disease, whether CNS disease was present or not (P = .92). Although some patients had CNS radiation, there was no evidence that this was of therapeutic benefit. Intrathecal (IT) chemoprophylaxis effectively prevented spread to the CNS in patients without initial CNS involvement. Five of 18 patients (28%) who received no IT prophylaxis had CNS relapse (four isolated to the CNS), but only seven of the 85 patients (8%) who received IT prophylaxis had CNS relapse (two isolated to the CNS). The differences in overall and isolated CNS relapse rates were statistically significant (P = .034 and P = .008, respectively).

Leptomeningeal Precursor B-Cell Lymphoblastic Lymphoma in a Child With Minimal Bone Marrow Involvement

2004 ◽  
Vol 26 (7) ◽  
pp. 469-472 ◽  
Author(s):  
Oussama Abla ◽  
Ahmed Naqvi ◽  
Charles Ye ◽  
Rakesh Bhattacharjee ◽  
Mary Shago ◽  
...  
Keyword(s):  
Bone Marrow ◽  
B Cell ◽  
Bone Marrow Involvement ◽  
Lymphoblastic Lymphoma

Allogeneic bone marrow transplantation for acute lymphoblastic leukemia in remission: prolonged survival associated with acute graft-versus-host disease.

1987 ◽  
Vol 5 (9) ◽  
pp. 1348-1355 ◽  
Author(s):  
D J Weisdorf ◽  
M E Nesbit ◽  
N K Ramsay ◽  
W G Woods ◽  
A I Goldman ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Acute Lymphoblastic Leukemia ◽  
Bone Marrow Transplantation ◽  
High Risk ◽  
Marrow Transplantation ◽  
Lymphoblastic Leukemia ◽  
Maintenance Chemotherapy ◽  
Relapse Free Survival ◽  
Free Survival ◽  
Acute Graft

Forty remission patients with high-risk acute lymphoblastic leukemia (ALL) underwent matched allogenic bone marrow transplantation (BMT) following preparation with cyclophosphamide and fractionated total body irradiation (TBI). As of March 1987, the median follow-up is more than 3 1/2 years. Thirteen patients are alive (11 relapse free) between 2 and 4 1/2 years post-BMT. Neither age, sex, remission number, prior extramedullary leukemia, nor WBC at diagnosis of ALL was statistically significant as a predictor of relapse-free survival. The development of acute graft-v-host disease (GVHD) in 17 patients was found, with time-dependent Cox regression analysis, to be associated with a significant reduction in post-BMT relapse risk (P = .04) and improved disease-free survival (P = .11). A prospective, randomized trial of maintenance chemotherapy with oral methotrexate and mercaptopurine did not demonstrate improvement in relapse risk or survival for those assigned maintenance chemotherapy (P = .7). These results suggest that allogeneic BMT can result in extended relapse-free survival for some patients with high-risk ALL. More effective preparative chemoradiotherapy and exploitation of the apparent graft-v-leukemia effect may be useful in future trials.

Autologous versus allogeneic bone marrow transplantation for non-Hodgkin's lymphoma: a case-controlled analysis of the European Bone Marrow Transplant Group Registry data.

1992 ◽  
Vol 10 (11) ◽  
pp. 1690-1695 ◽  
Author(s):  
R Chopra ◽  
A H Goldstone ◽  
R Pearce ◽  
T Philip ◽  
F Petersen ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Hodgkin’S Lymphoma ◽  
Progression Free Survival ◽  
Lymphoblastic Lymphoma ◽  
Hodgkin's Lymphoma ◽  
Controlled Study ◽  
Progression Rate ◽  
Free Survival ◽  
Non Hodgkin’S Lymphoma ◽  
Non Hodgkin's Lymphoma

PURPOSE A case-controlled study of patients who reported to the European Bone Marrow Transplant Group (EBMTG) was performed to investigate the relative roles and efficacy of allogeneic (alloBMT) and autologous bone marrow transplantation (ABMT) in non-Hodgkin's lymphoma. PATIENTS AND METHODS Of 1,060 patients who reported to the lymphoma registry, 938 patients underwent ABMT and 122 patients underwent alloBMT. A case-controlled study was performed by matching 101 alloBMT patients with 101 ABMT patients. The case matching was performed after the selection of the main prognostic factors for progression-free survival by a multivariate analysis. RESULTS The progression-free survival was similar in both types of transplants (49% alloBMT v 46% ABMT). The overall relapse and progression rate for the alloBMT patients was 23% compared with 38% in the ABMT patients. This difference was not significant statistically. In the lymphoblastic lymphoma subgroup, alloBMT was associated with a lower relapse rate than ABMT (24% alloBMT v 48% ABMT; P = .035). The progression-free survival, however, was not significantly different because patients with lymphoblastic lymphoma who underwent alloBMT had a higher procedure-related mortality (24% alloBMT v 10% ABMT; P = .06). A significantly lower relapse/progression rate was also observed in patients with chronic graft-versus-host disease (cGVHD) compared with those patients without (0% cGVHD v 35% no cGVHD; P = .02). Fourteen of 18 patients who had cGVHD also had lymphoblastic lymphoma. CONCLUSION This study suggests that ABMT and alloBMT for non-Hodgkin's lymphoma are comparable, with the exception of lymphoblastic lymphoma in which a graft-versus-lymphoma effect may account for the lower relapse rate for patients who underwent alloBMT.

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