Intensive chemotherapy for systemic anaplastic large cell lymphoma in children and adolescents: Final results of children's cancer group study 5941

Eric J. Lowe, Richard Sposto, Sherrie L. Perkins, Thomas G. Gross, Jonathan Finlay, David Zwick, Minnie Abromowitch

Research output: Contribution to journalArticle

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Abstract

Background. Anaplastic large cell lymphoma (ALCL) is characterized by advanced disease at presentation (70-80% of pediatric cases) and accounts for 10-15% of all childhood lymphomas. Treatment strategies for pediatric ALCL vary from short pulse B-NHL chemotherapy to prolonged leukemia like therapy. The optimal treatment strategy is unknown. Methods. CCG-5941 used a compressed aggressive multiagent T-cell lineage chemotherapy regimen consisting of a 3-week induction therapy (vincristine, prednisone, cyclophosphamide, daunomycin, asparaginase) followed by a 3-week consolidation period (vincristine, prednisone, etoposide, 6-thioguanine, cytarabine, asparaginase, methotrexate) followed by six courses of maintenance chemotherapy at 7-week intervals (cyclophosphamide, 6-thioguanine, vincristine, predni- sone, doxorubicin, asparaginase, methotrexate etoposide, cytara- bine). Total therapy was 48 weeks. Results. Eighty-six children (male 56%, female 44%) with non-localized ALCL (CD30+) were treated. The majority of tumors were positive for ALK (90%) and of T lineage (83%). Extranodal disease was common (mediastinum 35%, skin 15%, lung 14%, bone 12%, bone marrow 13%, liver 6%, and other viscera 17%). Grade 4 neutropenia occurred in 82% of patients. The 5-year EFS was 68% (95% CI of 57-78%) and the 5-year OS was 80% (95% CI of 69-87%). There were 21 relapses and 4 toxic deaths as first events. Relapse occurred early with 17 (81%) relapses occurring within 2 years of diagnosis and 12 (57%) while receiving therapy. Univariate analysis for risk factors only identified bone marrow involvement predicting lower EFS (P = 0.03). Conclusions. CCG-5941 demonstrated efficacy similar to previously reported regimens but with significant hematologic toxicity.

Original languageEnglish (US)
Pages (from-to)335-339
Number of pages5
JournalPediatric Blood and Cancer
Volume52
Issue number3
DOIs
StatePublished - Mar 1 2009

Fingerprint

Anaplastic Large-Cell Lymphoma
Asparaginase
Drug Therapy
Vincristine
Prednisone
Thioguanine
Neoplasms
Cytarabine
Etoposide
Methotrexate
Recurrence
Cyclophosphamide
Therapeutics
Bone Marrow
Maintenance Chemotherapy
Pediatrics
Daunorubicin
Viscera
Poisons
Mediastinum

Keywords

  • ALK positive lymphoma
  • Anaplastic large cell lymphoma
  • CD30
  • Non-Hodgkin lymphoma
  • Pediatric;t(2 ;5)

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

Cite this

Intensive chemotherapy for systemic anaplastic large cell lymphoma in children and adolescents : Final results of children's cancer group study 5941. / Lowe, Eric J.; Sposto, Richard; Perkins, Sherrie L.; Gross, Thomas G.; Finlay, Jonathan; Zwick, David; Abromowitch, Minnie.

In: Pediatric Blood and Cancer, Vol. 52, No. 3, 01.03.2009, p. 335-339.

Research output: Contribution to journalArticle

Lowe, Eric J. ; Sposto, Richard ; Perkins, Sherrie L. ; Gross, Thomas G. ; Finlay, Jonathan ; Zwick, David ; Abromowitch, Minnie. / Intensive chemotherapy for systemic anaplastic large cell lymphoma in children and adolescents : Final results of children's cancer group study 5941. In: Pediatric Blood and Cancer. 2009 ; Vol. 52, No. 3. pp. 335-339.
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abstract = "Background. Anaplastic large cell lymphoma (ALCL) is characterized by advanced disease at presentation (70-80{\%} of pediatric cases) and accounts for 10-15{\%} of all childhood lymphomas. Treatment strategies for pediatric ALCL vary from short pulse B-NHL chemotherapy to prolonged leukemia like therapy. The optimal treatment strategy is unknown. Methods. CCG-5941 used a compressed aggressive multiagent T-cell lineage chemotherapy regimen consisting of a 3-week induction therapy (vincristine, prednisone, cyclophosphamide, daunomycin, asparaginase) followed by a 3-week consolidation period (vincristine, prednisone, etoposide, 6-thioguanine, cytarabine, asparaginase, methotrexate) followed by six courses of maintenance chemotherapy at 7-week intervals (cyclophosphamide, 6-thioguanine, vincristine, predni- sone, doxorubicin, asparaginase, methotrexate etoposide, cytara- bine). Total therapy was 48 weeks. Results. Eighty-six children (male 56{\%}, female 44{\%}) with non-localized ALCL (CD30+) were treated. The majority of tumors were positive for ALK (90{\%}) and of T lineage (83{\%}). Extranodal disease was common (mediastinum 35{\%}, skin 15{\%}, lung 14{\%}, bone 12{\%}, bone marrow 13{\%}, liver 6{\%}, and other viscera 17{\%}). Grade 4 neutropenia occurred in 82{\%} of patients. The 5-year EFS was 68{\%} (95{\%} CI of 57-78{\%}) and the 5-year OS was 80{\%} (95{\%} CI of 69-87{\%}). There were 21 relapses and 4 toxic deaths as first events. Relapse occurred early with 17 (81{\%}) relapses occurring within 2 years of diagnosis and 12 (57{\%}) while receiving therapy. Univariate analysis for risk factors only identified bone marrow involvement predicting lower EFS (P = 0.03). Conclusions. CCG-5941 demonstrated efficacy similar to previously reported regimens but with significant hematologic toxicity.",
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AU - Lowe, Eric J.

AU - Sposto, Richard

AU - Perkins, Sherrie L.

AU - Gross, Thomas G.

AU - Finlay, Jonathan

AU - Zwick, David

AU - Abromowitch, Minnie

PY - 2009/3/1

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N2 - Background. Anaplastic large cell lymphoma (ALCL) is characterized by advanced disease at presentation (70-80% of pediatric cases) and accounts for 10-15% of all childhood lymphomas. Treatment strategies for pediatric ALCL vary from short pulse B-NHL chemotherapy to prolonged leukemia like therapy. The optimal treatment strategy is unknown. Methods. CCG-5941 used a compressed aggressive multiagent T-cell lineage chemotherapy regimen consisting of a 3-week induction therapy (vincristine, prednisone, cyclophosphamide, daunomycin, asparaginase) followed by a 3-week consolidation period (vincristine, prednisone, etoposide, 6-thioguanine, cytarabine, asparaginase, methotrexate) followed by six courses of maintenance chemotherapy at 7-week intervals (cyclophosphamide, 6-thioguanine, vincristine, predni- sone, doxorubicin, asparaginase, methotrexate etoposide, cytara- bine). Total therapy was 48 weeks. Results. Eighty-six children (male 56%, female 44%) with non-localized ALCL (CD30+) were treated. The majority of tumors were positive for ALK (90%) and of T lineage (83%). Extranodal disease was common (mediastinum 35%, skin 15%, lung 14%, bone 12%, bone marrow 13%, liver 6%, and other viscera 17%). Grade 4 neutropenia occurred in 82% of patients. The 5-year EFS was 68% (95% CI of 57-78%) and the 5-year OS was 80% (95% CI of 69-87%). There were 21 relapses and 4 toxic deaths as first events. Relapse occurred early with 17 (81%) relapses occurring within 2 years of diagnosis and 12 (57%) while receiving therapy. Univariate analysis for risk factors only identified bone marrow involvement predicting lower EFS (P = 0.03). Conclusions. CCG-5941 demonstrated efficacy similar to previously reported regimens but with significant hematologic toxicity.

AB - Background. Anaplastic large cell lymphoma (ALCL) is characterized by advanced disease at presentation (70-80% of pediatric cases) and accounts for 10-15% of all childhood lymphomas. Treatment strategies for pediatric ALCL vary from short pulse B-NHL chemotherapy to prolonged leukemia like therapy. The optimal treatment strategy is unknown. Methods. CCG-5941 used a compressed aggressive multiagent T-cell lineage chemotherapy regimen consisting of a 3-week induction therapy (vincristine, prednisone, cyclophosphamide, daunomycin, asparaginase) followed by a 3-week consolidation period (vincristine, prednisone, etoposide, 6-thioguanine, cytarabine, asparaginase, methotrexate) followed by six courses of maintenance chemotherapy at 7-week intervals (cyclophosphamide, 6-thioguanine, vincristine, predni- sone, doxorubicin, asparaginase, methotrexate etoposide, cytara- bine). Total therapy was 48 weeks. Results. Eighty-six children (male 56%, female 44%) with non-localized ALCL (CD30+) were treated. The majority of tumors were positive for ALK (90%) and of T lineage (83%). Extranodal disease was common (mediastinum 35%, skin 15%, lung 14%, bone 12%, bone marrow 13%, liver 6%, and other viscera 17%). Grade 4 neutropenia occurred in 82% of patients. The 5-year EFS was 68% (95% CI of 57-78%) and the 5-year OS was 80% (95% CI of 69-87%). There were 21 relapses and 4 toxic deaths as first events. Relapse occurred early with 17 (81%) relapses occurring within 2 years of diagnosis and 12 (57%) while receiving therapy. Univariate analysis for risk factors only identified bone marrow involvement predicting lower EFS (P = 0.03). Conclusions. CCG-5941 demonstrated efficacy similar to previously reported regimens but with significant hematologic toxicity.

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