Precursor B-cell lymphoblastic lymphoma in childhood and adolescence: Clinical features, treatment, and results in trials NHL-BFM 86 and 90

Background Precursor B‐cell lymphoblastic lymphoma (PBLL) is a rare subtype of childhood non‐Hodgkin lymphoma (NHL). The purpose of our study was to investigate frequency and clinicopathological features of PBLL in children and to test prospectively the efficacy of an ALL‐type therapy for treatment...

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Veröffentlicht in:Medical and pediatric oncology 2000-07, Vol.35 (1), p.20-27
Hauptverfasser: Neth, Olaf, Seidemann, Kathrin, Jansen, Petra, Mann, Georg, Tiemann, Markus, Ludwig, Wolf-Dieter, Riehm, Hansjörg, Reiter, Alfred
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container_issue 1
container_start_page 20
container_title Medical and pediatric oncology
container_volume 35
creator Neth, Olaf
Seidemann, Kathrin
Jansen, Petra
Mann, Georg
Tiemann, Markus
Ludwig, Wolf-Dieter
Riehm, Hansjörg
Reiter, Alfred
description Background Precursor B‐cell lymphoblastic lymphoma (PBLL) is a rare subtype of childhood non‐Hodgkin lymphoma (NHL). The purpose of our study was to investigate frequency and clinicopathological features of PBLL in children and to test prospectively the efficacy of an ALL‐type therapy for treatment of these patients. Procedure From October, 1986, to March, 1995, 1,075 patients up to 18 years of age suffering from all kinds of NHL were registered in the two consecutive multicenter studies NHL‐BFM 86 and 90. Of these, 27 patients were diagnosed with PBLL. Twenty‐one PBLL patients were treated according to a BFM‐ALL‐type protocol: an eight‐drug induction over 9 weeks was followed by an 8‐week consolidation including methotrexate 5 g/m2 ×4. Patients in stages I and II continued with maintenance up to a total therapy duration of 24 months, whereas patients in stages III and IV received an additional eight‐drug intensification and cranial radiotherapy (12 Gy for prophylaxis) after consolidation. Six PBLL patients were treated according to the BFM‐protocol for B‐NHL, stratified according to stage and tumor load and consisiting of two to six 5‐day courses of chemotherapy. Results The median age of the 27 patients with PBLL (18 boys, 9 girls) was 6.2 (range 0.7–15) years. Stages (St. Jude) were: I (n = 3), II (n = 7), III (n = 9), and IV (n = 8). Twenty‐one PBLL patients had nodal disease, 6 patients had subcutaneous manifestations, and 8 patients had bone marrow disease (
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The purpose of our study was to investigate frequency and clinicopathological features of PBLL in children and to test prospectively the efficacy of an ALL‐type therapy for treatment of these patients. Procedure From October, 1986, to March, 1995, 1,075 patients up to 18 years of age suffering from all kinds of NHL were registered in the two consecutive multicenter studies NHL‐BFM 86 and 90. Of these, 27 patients were diagnosed with PBLL. Twenty‐one PBLL patients were treated according to a BFM‐ALL‐type protocol: an eight‐drug induction over 9 weeks was followed by an 8‐week consolidation including methotrexate 5 g/m2 ×4. Patients in stages I and II continued with maintenance up to a total therapy duration of 24 months, whereas patients in stages III and IV received an additional eight‐drug intensification and cranial radiotherapy (12 Gy for prophylaxis) after consolidation. Six PBLL patients were treated according to the BFM‐protocol for B‐NHL, stratified according to stage and tumor load and consisiting of two to six 5‐day courses of chemotherapy. Results The median age of the 27 patients with PBLL (18 boys, 9 girls) was 6.2 (range 0.7–15) years. Stages (St. Jude) were: I (n = 3), II (n = 7), III (n = 9), and IV (n = 8). Twenty‐one PBLL patients had nodal disease, 6 patients had subcutaneous manifestations, and 8 patients had bone marrow disease (&lt;25% blasts). All patients achieved remission. With a median follow‐up time of 4.25 years, the estimated probability for event‐free survival (pEFS) at 10 years for the total group was 0.73 (SE 0.10). Five patients (2, 1, 1, and 1 patients at stages I, II, III, and IV, respectively) relapsed: 2 of 21 patients who were treated according to the ALL strategy and 3 of 6 who were treated according to the B‐NHL‐protocol. Conclusions PBLL accounts for 2.5% of childhood NHL. An ALL‐type therapy strategy appears to be superior to a short‐pulse B‐NHL protocol. Med. Pediatr. Oncol. 35:20–27, 2000. © 2000 Wiley‐Liss, Inc.</description><identifier>ISSN: 0098-1532</identifier><identifier>EISSN: 1096-911X</identifier><identifier>DOI: 10.1002/1096-911X(200007)35:1&lt;20::AID-MPO4&gt;3.0.CO;2-L</identifier><identifier>PMID: 10881003</identifier><identifier>CODEN: MPONDB</identifier><language>eng</language><publisher>New York: John Wiley &amp; Sons, Inc</publisher><subject>Adolescent ; Antineoplastic agents ; Antineoplastic Combined Chemotherapy Protocols - therapeutic use ; Asparaginase - administration &amp; dosage ; Austria - epidemiology ; B-Lymphocytes ; Biological and medical sciences ; Chemotherapy ; Child ; Child, Preschool ; childhood therapy ; Cohort Studies ; Daunorubicin - administration &amp; dosage ; Disease-Free Survival ; Female ; Germany - epidemiology ; Humans ; Infant ; lymphoblastic lymphoma ; Male ; Medical sciences ; Multicenter Studies as Topic ; Neoplasm Staging ; Pharmacology. Drug treatments ; precursor B-cell ; Precursor Cell Lymphoblastic Leukemia-Lymphoma - drug therapy ; Precursor Cell Lymphoblastic Leukemia-Lymphoma - mortality ; Prednisone - administration &amp; dosage ; Prospective Studies ; Registries ; Survival Analysis ; Vincristine - administration &amp; dosage</subject><ispartof>Medical and pediatric oncology, 2000-07, Vol.35 (1), p.20-27</ispartof><rights>Copyright © 2000 Wiley‐Liss, Inc.</rights><rights>2000 INIST-CNRS</rights><rights>Copyright 2000 Wiley-Liss, Inc.</rights><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c4814-62a2fcbcbd7e0d1cbdae1f71181b68200f1701c6b735d56829792ac0aae44f4f3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1002%2F1096-911X%28200007%2935%3A1%3C20%3A%3AAID-MPO4%3E3.0.CO%3B2-L$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1002%2F1096-911X%28200007%2935%3A1%3C20%3A%3AAID-MPO4%3E3.0.CO%3B2-L$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,780,784,1417,27923,27924,45573,45574</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&amp;idt=1536018$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/10881003$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Neth, Olaf</creatorcontrib><creatorcontrib>Seidemann, Kathrin</creatorcontrib><creatorcontrib>Jansen, Petra</creatorcontrib><creatorcontrib>Mann, Georg</creatorcontrib><creatorcontrib>Tiemann, Markus</creatorcontrib><creatorcontrib>Ludwig, Wolf-Dieter</creatorcontrib><creatorcontrib>Riehm, Hansjörg</creatorcontrib><creatorcontrib>Reiter, Alfred</creatorcontrib><title>Precursor B-cell lymphoblastic lymphoma in childhood and adolescence: Clinical features, treatment, and results in trials NHL-BFM 86 and 90</title><title>Medical and pediatric oncology</title><addtitle>Med. Pediatr. Oncol</addtitle><description>Background Precursor B‐cell lymphoblastic lymphoma (PBLL) is a rare subtype of childhood non‐Hodgkin lymphoma (NHL). The purpose of our study was to investigate frequency and clinicopathological features of PBLL in children and to test prospectively the efficacy of an ALL‐type therapy for treatment of these patients. Procedure From October, 1986, to March, 1995, 1,075 patients up to 18 years of age suffering from all kinds of NHL were registered in the two consecutive multicenter studies NHL‐BFM 86 and 90. Of these, 27 patients were diagnosed with PBLL. Twenty‐one PBLL patients were treated according to a BFM‐ALL‐type protocol: an eight‐drug induction over 9 weeks was followed by an 8‐week consolidation including methotrexate 5 g/m2 ×4. Patients in stages I and II continued with maintenance up to a total therapy duration of 24 months, whereas patients in stages III and IV received an additional eight‐drug intensification and cranial radiotherapy (12 Gy for prophylaxis) after consolidation. Six PBLL patients were treated according to the BFM‐protocol for B‐NHL, stratified according to stage and tumor load and consisiting of two to six 5‐day courses of chemotherapy. Results The median age of the 27 patients with PBLL (18 boys, 9 girls) was 6.2 (range 0.7–15) years. Stages (St. Jude) were: I (n = 3), II (n = 7), III (n = 9), and IV (n = 8). Twenty‐one PBLL patients had nodal disease, 6 patients had subcutaneous manifestations, and 8 patients had bone marrow disease (&lt;25% blasts). All patients achieved remission. With a median follow‐up time of 4.25 years, the estimated probability for event‐free survival (pEFS) at 10 years for the total group was 0.73 (SE 0.10). Five patients (2, 1, 1, and 1 patients at stages I, II, III, and IV, respectively) relapsed: 2 of 21 patients who were treated according to the ALL strategy and 3 of 6 who were treated according to the B‐NHL‐protocol. Conclusions PBLL accounts for 2.5% of childhood NHL. An ALL‐type therapy strategy appears to be superior to a short‐pulse B‐NHL protocol. Med. Pediatr. Oncol. 35:20–27, 2000. © 2000 Wiley‐Liss, Inc.</description><subject>Adolescent</subject><subject>Antineoplastic agents</subject><subject>Antineoplastic Combined Chemotherapy Protocols - therapeutic use</subject><subject>Asparaginase - administration &amp; dosage</subject><subject>Austria - epidemiology</subject><subject>B-Lymphocytes</subject><subject>Biological and medical sciences</subject><subject>Chemotherapy</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>childhood therapy</subject><subject>Cohort Studies</subject><subject>Daunorubicin - administration &amp; dosage</subject><subject>Disease-Free Survival</subject><subject>Female</subject><subject>Germany - epidemiology</subject><subject>Humans</subject><subject>Infant</subject><subject>lymphoblastic lymphoma</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Multicenter Studies as Topic</subject><subject>Neoplasm Staging</subject><subject>Pharmacology. Drug treatments</subject><subject>precursor B-cell</subject><subject>Precursor Cell Lymphoblastic Leukemia-Lymphoma - drug therapy</subject><subject>Precursor Cell Lymphoblastic Leukemia-Lymphoma - mortality</subject><subject>Prednisone - administration &amp; dosage</subject><subject>Prospective Studies</subject><subject>Registries</subject><subject>Survival Analysis</subject><subject>Vincristine - administration &amp; dosage</subject><issn>0098-1532</issn><issn>1096-911X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2000</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNkl1v0zAUhi0EYmXwF1AuEGLSUvyRxElBk7bAPqS03QVoaDdHjuOoBicpdiLob-BP4zTV4IILLFm2jx-_Pj6vEYoJnhOM6VuCsyTMCPnyhmLf-AmLF-Q9xYvF-c2HcHm7js7YHM_z9TsaFo_Q7IF_jGYYZ2lIYkaP0DPnvvrjWcbTp-iI4DT16myGft1aJQfrOhtchFIZE5hds910pRGu1_KwakSg20ButKk2XVcFovW96oxyUrVSLYLc6FZLYYJaiX6wyp0GvfXTRrX96R73scH0btTprRbGBavrIry4XAZpsgcy_Bw9qf2GenEYj9Hny4-f8uuwWF_d5OdFKKOURGFCBa1lKcuKK1wRPwpFak5ISsok9VWqCcdEJiVncRX7SMYzKiQWQkVRHdXsGL2edLe2-z4o10Oj3fh20apucMAJZRzj2IPLCZS2c86qGrZWN8LugGAY3YGx2jBWGyZ3gMVA_BzAuwOjO8AAQ74GCoXXe3m4eCgbVf2lNtnhgVcHQDhfzdqKVmr3h4tZgknqsdWE_dBG7f4_qX_ktF97wXAS1K5XPx8Ehf0GCWc8hrvVFYy_5764o3DPfgOQkcL4</recordid><startdate>200007</startdate><enddate>200007</enddate><creator>Neth, Olaf</creator><creator>Seidemann, Kathrin</creator><creator>Jansen, Petra</creator><creator>Mann, Georg</creator><creator>Tiemann, Markus</creator><creator>Ludwig, Wolf-Dieter</creator><creator>Riehm, Hansjörg</creator><creator>Reiter, Alfred</creator><general>John Wiley &amp; Sons, Inc</general><general>Wiley-Liss</general><scope>BSCLL</scope><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>200007</creationdate><title>Precursor B-cell lymphoblastic lymphoma in childhood and adolescence: Clinical features, treatment, and results in trials NHL-BFM 86 and 90</title><author>Neth, Olaf ; Seidemann, Kathrin ; Jansen, Petra ; Mann, Georg ; Tiemann, Markus ; Ludwig, Wolf-Dieter ; Riehm, Hansjörg ; Reiter, Alfred</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4814-62a2fcbcbd7e0d1cbdae1f71181b68200f1701c6b735d56829792ac0aae44f4f3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2000</creationdate><topic>Adolescent</topic><topic>Antineoplastic agents</topic><topic>Antineoplastic Combined Chemotherapy Protocols - therapeutic use</topic><topic>Asparaginase - administration &amp; dosage</topic><topic>Austria - epidemiology</topic><topic>B-Lymphocytes</topic><topic>Biological and medical sciences</topic><topic>Chemotherapy</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>childhood therapy</topic><topic>Cohort Studies</topic><topic>Daunorubicin - administration &amp; dosage</topic><topic>Disease-Free Survival</topic><topic>Female</topic><topic>Germany - epidemiology</topic><topic>Humans</topic><topic>Infant</topic><topic>lymphoblastic lymphoma</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Multicenter Studies as Topic</topic><topic>Neoplasm Staging</topic><topic>Pharmacology. Drug treatments</topic><topic>precursor B-cell</topic><topic>Precursor Cell Lymphoblastic Leukemia-Lymphoma - drug therapy</topic><topic>Precursor Cell Lymphoblastic Leukemia-Lymphoma - mortality</topic><topic>Prednisone - administration &amp; dosage</topic><topic>Prospective Studies</topic><topic>Registries</topic><topic>Survival Analysis</topic><topic>Vincristine - administration &amp; dosage</topic><toplevel>online_resources</toplevel><creatorcontrib>Neth, Olaf</creatorcontrib><creatorcontrib>Seidemann, Kathrin</creatorcontrib><creatorcontrib>Jansen, Petra</creatorcontrib><creatorcontrib>Mann, Georg</creatorcontrib><creatorcontrib>Tiemann, Markus</creatorcontrib><creatorcontrib>Ludwig, Wolf-Dieter</creatorcontrib><creatorcontrib>Riehm, Hansjörg</creatorcontrib><creatorcontrib>Reiter, Alfred</creatorcontrib><collection>Istex</collection><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Medical and pediatric oncology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Neth, Olaf</au><au>Seidemann, Kathrin</au><au>Jansen, Petra</au><au>Mann, Georg</au><au>Tiemann, Markus</au><au>Ludwig, Wolf-Dieter</au><au>Riehm, Hansjörg</au><au>Reiter, Alfred</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Precursor B-cell lymphoblastic lymphoma in childhood and adolescence: Clinical features, treatment, and results in trials NHL-BFM 86 and 90</atitle><jtitle>Medical and pediatric oncology</jtitle><addtitle>Med. Pediatr. Oncol</addtitle><date>2000-07</date><risdate>2000</risdate><volume>35</volume><issue>1</issue><spage>20</spage><epage>27</epage><pages>20-27</pages><issn>0098-1532</issn><eissn>1096-911X</eissn><coden>MPONDB</coden><abstract>Background Precursor B‐cell lymphoblastic lymphoma (PBLL) is a rare subtype of childhood non‐Hodgkin lymphoma (NHL). The purpose of our study was to investigate frequency and clinicopathological features of PBLL in children and to test prospectively the efficacy of an ALL‐type therapy for treatment of these patients. Procedure From October, 1986, to March, 1995, 1,075 patients up to 18 years of age suffering from all kinds of NHL were registered in the two consecutive multicenter studies NHL‐BFM 86 and 90. Of these, 27 patients were diagnosed with PBLL. Twenty‐one PBLL patients were treated according to a BFM‐ALL‐type protocol: an eight‐drug induction over 9 weeks was followed by an 8‐week consolidation including methotrexate 5 g/m2 ×4. Patients in stages I and II continued with maintenance up to a total therapy duration of 24 months, whereas patients in stages III and IV received an additional eight‐drug intensification and cranial radiotherapy (12 Gy for prophylaxis) after consolidation. Six PBLL patients were treated according to the BFM‐protocol for B‐NHL, stratified according to stage and tumor load and consisiting of two to six 5‐day courses of chemotherapy. Results The median age of the 27 patients with PBLL (18 boys, 9 girls) was 6.2 (range 0.7–15) years. Stages (St. Jude) were: I (n = 3), II (n = 7), III (n = 9), and IV (n = 8). Twenty‐one PBLL patients had nodal disease, 6 patients had subcutaneous manifestations, and 8 patients had bone marrow disease (&lt;25% blasts). All patients achieved remission. With a median follow‐up time of 4.25 years, the estimated probability for event‐free survival (pEFS) at 10 years for the total group was 0.73 (SE 0.10). Five patients (2, 1, 1, and 1 patients at stages I, II, III, and IV, respectively) relapsed: 2 of 21 patients who were treated according to the ALL strategy and 3 of 6 who were treated according to the B‐NHL‐protocol. Conclusions PBLL accounts for 2.5% of childhood NHL. An ALL‐type therapy strategy appears to be superior to a short‐pulse B‐NHL protocol. Med. Pediatr. Oncol. 35:20–27, 2000. © 2000 Wiley‐Liss, Inc.</abstract><cop>New York</cop><pub>John Wiley &amp; Sons, Inc</pub><pmid>10881003</pmid><doi>10.1002/1096-911X(200007)35:1&lt;20::AID-MPO4&gt;3.0.CO;2-L</doi><tpages>8</tpages></addata></record>
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source MEDLINE; Wiley Online Library Journals Frontfile Complete
subjects Adolescent
Antineoplastic agents
Antineoplastic Combined Chemotherapy Protocols - therapeutic use
Asparaginase - administration & dosage
Austria - epidemiology
B-Lymphocytes
Biological and medical sciences
Chemotherapy
Child
Child, Preschool
childhood therapy
Cohort Studies
Daunorubicin - administration & dosage
Disease-Free Survival
Female
Germany - epidemiology
Humans
Infant
lymphoblastic lymphoma
Male
Medical sciences
Multicenter Studies as Topic
Neoplasm Staging
Pharmacology. Drug treatments
precursor B-cell
Precursor Cell Lymphoblastic Leukemia-Lymphoma - drug therapy
Precursor Cell Lymphoblastic Leukemia-Lymphoma - mortality
Prednisone - administration & dosage
Prospective Studies
Registries
Survival Analysis
Vincristine - administration & dosage
title Precursor B-cell lymphoblastic lymphoma in childhood and adolescence: Clinical features, treatment, and results in trials NHL-BFM 86 and 90
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