In childhood mature B-NHL with CNS disease, patients with blasts in cerebrospinal fluid are at higher risk of failure

To identify the factors influencing outcome in childhood mature B-cell non-Hodgkin lymphoma and acute leukemia (B-NHL/AL) with central nervous system (CNS) disease (CNS+), we analyzed patients

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Veröffentlicht in:Blood advances 2020-08, Vol.4 (15), p.3621-3625
Hauptverfasser: Simonin, Mathieu, Auperin, Anne, Bertrand, Yves, Aladjidi, Nathalie, Baruchel, André, Contet, Audrey, Coze, Carole, Gandemer, Virginie, Haouy, Stephanie, Leblanc, Thierry, Leverger, Guy, Michon, Jean, Patte, Catherine, Minard-Colin, Veronique
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container_end_page 3625
container_issue 15
container_start_page 3621
container_title Blood advances
container_volume 4
creator Simonin, Mathieu
Auperin, Anne
Bertrand, Yves
Aladjidi, Nathalie
Baruchel, André
Contet, Audrey
Coze, Carole
Gandemer, Virginie
Haouy, Stephanie
Leblanc, Thierry
Leverger, Guy
Michon, Jean
Patte, Catherine
Minard-Colin, Veronique
description To identify the factors influencing outcome in childhood mature B-cell non-Hodgkin lymphoma and acute leukemia (B-NHL/AL) with central nervous system (CNS) disease (CNS+), we analyzed patients
doi_str_mv 10.1182/bloodadvances.2019001398
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CNS+ was diagnosed on fulfillment of ≥1 of the following criteria: any L3 cerebrospinal fluid (CSF) blasts (CSF+), cranial nerve palsy, isolated intracerebral mass but also clinical spinal cord compression, and cranial or spinal parameningeal extension. Two hundred seventeen out of 1690 patients (12.8%) were CNS+. CNS+ was significantly associated with male gender, head/neck locations, Burkitt histology, high initial lactate dehydrogenase (LDH) level, and bone marrow involvement. CSF+ was the most frequent pattern of CNS+ (45%). For the 217 CNS+ patients, the 5-year event-free survival (EFS) and overall survival rates (95% confidence interval) were 81.5% (75.8% to 86.1%) and 83.9% (78.4% to 88.2%), respectively. In multivariate analysis, among CNS+ patients, low EFS was associated with CSF+, high initial LDH level, and poor response to cyclophosphamide, oncovin (vincristine), prednisone prephase. These findings have been considered for patient's stratification in the international randomized phase 3 trial Inter-B-NHL-ritux 2010 for children and adolescents with high-risk B-NHL/AL with CNS+ CSF+ patients only receiving intensified chemotherapy. •Largest cohort of childhood mature B-NHL/AL with CNS disease reporting prevalence, clinical pattern, and outcome.•In patients with CNS disease, those with blasts in the CSF or LDH value above twice the upper normal value are at higher risk of failure. 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CNS+ was diagnosed on fulfillment of ≥1 of the following criteria: any L3 cerebrospinal fluid (CSF) blasts (CSF+), cranial nerve palsy, isolated intracerebral mass but also clinical spinal cord compression, and cranial or spinal parameningeal extension. Two hundred seventeen out of 1690 patients (12.8%) were CNS+. CNS+ was significantly associated with male gender, head/neck locations, Burkitt histology, high initial lactate dehydrogenase (LDH) level, and bone marrow involvement. CSF+ was the most frequent pattern of CNS+ (45%). For the 217 CNS+ patients, the 5-year event-free survival (EFS) and overall survival rates (95% confidence interval) were 81.5% (75.8% to 86.1%) and 83.9% (78.4% to 88.2%), respectively. In multivariate analysis, among CNS+ patients, low EFS was associated with CSF+, high initial LDH level, and poor response to cyclophosphamide, oncovin (vincristine), prednisone prephase. These findings have been considered for patient's stratification in the international randomized phase 3 trial Inter-B-NHL-ritux 2010 for children and adolescents with high-risk B-NHL/AL with CNS+ CSF+ patients only receiving intensified chemotherapy. •Largest cohort of childhood mature B-NHL/AL with CNS disease reporting prevalence, clinical pattern, and outcome.•In patients with CNS disease, those with blasts in the CSF or LDH value above twice the upper normal value are at higher risk of failure. 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subjects Adolescent
Antineoplastic Combined Chemotherapy Protocols
Central Nervous System Diseases
Child
Disease-Free Survival
Humans
Lymphoid Neoplasia
Lymphoma, B-Cell - drug therapy
Lymphoma, Non-Hodgkin
Male
title In childhood mature B-NHL with CNS disease, patients with blasts in cerebrospinal fluid are at higher risk of failure
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