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

70 Citations (Scopus)

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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