Poor prognosis for P2RY8-CRLF2 fusion but not for CRLF2 over-expression in children with intermediate risk B-cell precursor acute lymphoblastic leukemia
Pediatric B-cell precursor acute lymphoblastic leukemia (BCP-ALL) has achieved an 80% cure rate as a result of a risk-adapted therapy largely based on minimal residual disease (MRD) monitoring. However, relapse is still the most frequent adverse event, occurring mainly in the patients with intermedi...
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| Veröffentlicht in: | Leukemia 2012-10, Vol.26 (10), p.2245-2253 |
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| creator | Palmi, C Vendramini, E Silvestri, D Longinotti, G Frison, D Cario, G Shochat, C Stanulla, M Rossi, V Di Meglio, A M Villa, T Giarin, E Fazio, G Leszl, A Schrappe, M Basso, G Biondi, A Izraeli, S Conter, V Valsecchi, M G Cazzaniga, G te Kronnie, G |
| description | Pediatric B-cell precursor acute lymphoblastic leukemia (BCP-ALL) has achieved an 80% cure rate as a result of a risk-adapted therapy largely based on minimal residual disease (MRD) monitoring. However, relapse is still the most frequent adverse event, occurring mainly in the patients with intermediate MRD levels (intermediate risk, IR), emphasizing the need for new prognostic markers. We analyzed the prognostic impact of cytokine receptor-like factor 2 (
CRLF2
) over-expression and
P2RY8-CRLF2
fusion in 464 BCP-ALL patients (not affected by Down syndrome and
BCR-ABL
negative) enrolled in the AIEOP-BFM ALL2000 study in Italy. In 22/464 (4.7%) samples, RQ-PCR showed
CRLF2
over-expression (⩾20 times higher than the overall median).
P2RY8-CRLF2
fusion was detected in 22/365 (6%) cases, with 10/22 cases also showing
CRLF2
over-expression.
P2RY8-CRLF2
fusion was the most relevant prognostic factor independent of
CRLF2
over-expression with a threefold increase in risk of relapse. Significantly, the cumulative incidence of relapse of the
P2RY8-CRLF2+
patients in the IR group was high (61.1%±12.9 vs 17.6%±2.6,
P |
| doi_str_mv | 10.1038/leu.2012.101 |
| format | Article |
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CRLF2
) over-expression and
P2RY8-CRLF2
fusion in 464 BCP-ALL patients (not affected by Down syndrome and
BCR-ABL
negative) enrolled in the AIEOP-BFM ALL2000 study in Italy. In 22/464 (4.7%) samples, RQ-PCR showed
CRLF2
over-expression (⩾20 times higher than the overall median).
P2RY8-CRLF2
fusion was detected in 22/365 (6%) cases, with 10/22 cases also showing
CRLF2
over-expression.
P2RY8-CRLF2
fusion was the most relevant prognostic factor independent of
CRLF2
over-expression with a threefold increase in risk of relapse. Significantly, the cumulative incidence of relapse of the
P2RY8-CRLF2+
patients in the IR group was high (61.1%±12.9 vs 17.6%±2.6,
P
<0.0001), similar to high-risk patients in AIEOP-BFM ALL2000 study. These results were confirmed in a cohort of patients treated in Germany. In conclusion,
P2RY8-CRLF2
identifies a subset of BCP-ALL patients currently stratified as IR that could be considered for treatment intensification.</description><identifier>ISSN: 0887-6924</identifier><identifier>EISSN: 1476-5551</identifier><identifier>DOI: 10.1038/leu.2012.101</identifier><identifier>PMID: 22484421</identifier><identifier>CODEN: LEUKED</identifier><language>eng</language><publisher>London: Nature Publishing Group UK</publisher><subject>692/53/2422 ; 692/699/67/1990/283 ; 692/699/67/2332 ; Acute lymphoblastic leukemia ; Acute lymphocytic leukemia ; BCR-ABL protein ; Biological and medical sciences ; Cancer Research ; Cellular signal transduction ; Chromosomes ; Critical Care Medicine ; Cytokines ; Down syndrome ; Down's syndrome ; Fusion protein ; Gene expression ; Gene Fusion ; Genetic aspects ; Hematologic and hematopoietic diseases ; Hematology ; Humans ; Impact analysis ; Intensive ; Internal Medicine ; Leukemia ; Leukemia in children ; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis ; Lymphatic leukemia ; Lymphoblastic leukemia in children ; Lymphocytes B ; Lymphocytic leukemia in children ; Medical prognosis ; Medical sciences ; Medicine ; Medicine & Public Health ; Minimal residual disease ; Mutation ; Oncology ; original-article ; Overexpression ; Patients ; Pediatrics ; Physiological aspects ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma - genetics ; Precursors ; Prognosis ; Proportional Hazards Models ; Receptors, Cytokine - genetics ; Receptors, Cytokine - physiology ; Receptors, Purinergic P2 - genetics ; Recurrence ; Risk ; Risk Factors ; Risk groups</subject><ispartof>Leukemia, 2012-10, Vol.26 (10), p.2245-2253</ispartof><rights>Macmillan Publishers Limited 2012</rights><rights>2015 INIST-CNRS</rights><rights>COPYRIGHT 2012 Nature Publishing Group</rights><rights>Copyright Nature Publishing Group Oct 2012</rights><rights>Macmillan Publishers Limited 2012.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c584t-b5a169ba30b8105bf6b3ce1febc9df8a745643dd61f83279e6ba2f3235901f833</citedby><cites>FETCH-LOGICAL-c584t-b5a169ba30b8105bf6b3ce1febc9df8a745643dd61f83279e6ba2f3235901f833</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=26442722$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/22484421$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Palmi, C</creatorcontrib><creatorcontrib>Vendramini, E</creatorcontrib><creatorcontrib>Silvestri, D</creatorcontrib><creatorcontrib>Longinotti, G</creatorcontrib><creatorcontrib>Frison, D</creatorcontrib><creatorcontrib>Cario, G</creatorcontrib><creatorcontrib>Shochat, C</creatorcontrib><creatorcontrib>Stanulla, M</creatorcontrib><creatorcontrib>Rossi, V</creatorcontrib><creatorcontrib>Di Meglio, A M</creatorcontrib><creatorcontrib>Villa, T</creatorcontrib><creatorcontrib>Giarin, E</creatorcontrib><creatorcontrib>Fazio, G</creatorcontrib><creatorcontrib>Leszl, A</creatorcontrib><creatorcontrib>Schrappe, M</creatorcontrib><creatorcontrib>Basso, G</creatorcontrib><creatorcontrib>Biondi, A</creatorcontrib><creatorcontrib>Izraeli, S</creatorcontrib><creatorcontrib>Conter, V</creatorcontrib><creatorcontrib>Valsecchi, M G</creatorcontrib><creatorcontrib>Cazzaniga, G</creatorcontrib><creatorcontrib>te Kronnie, G</creatorcontrib><title>Poor prognosis for P2RY8-CRLF2 fusion but not for CRLF2 over-expression in children with intermediate risk B-cell precursor acute lymphoblastic leukemia</title><title>Leukemia</title><addtitle>Leukemia</addtitle><addtitle>Leukemia</addtitle><description>Pediatric B-cell precursor acute lymphoblastic leukemia (BCP-ALL) has achieved an 80% cure rate as a result of a risk-adapted therapy largely based on minimal residual disease (MRD) monitoring. However, relapse is still the most frequent adverse event, occurring mainly in the patients with intermediate MRD levels (intermediate risk, IR), emphasizing the need for new prognostic markers. We analyzed the prognostic impact of cytokine receptor-like factor 2 (
CRLF2
) over-expression and
P2RY8-CRLF2
fusion in 464 BCP-ALL patients (not affected by Down syndrome and
BCR-ABL
negative) enrolled in the AIEOP-BFM ALL2000 study in Italy. In 22/464 (4.7%) samples, RQ-PCR showed
CRLF2
over-expression (⩾20 times higher than the overall median).
P2RY8-CRLF2
fusion was detected in 22/365 (6%) cases, with 10/22 cases also showing
CRLF2
over-expression.
P2RY8-CRLF2
fusion was the most relevant prognostic factor independent of
CRLF2
over-expression with a threefold increase in risk of relapse. Significantly, the cumulative incidence of relapse of the
P2RY8-CRLF2+
patients in the IR group was high (61.1%±12.9 vs 17.6%±2.6,
P
<0.0001), similar to high-risk patients in AIEOP-BFM ALL2000 study. These results were confirmed in a cohort of patients treated in Germany. In conclusion,
P2RY8-CRLF2
identifies a subset of BCP-ALL patients currently stratified as IR that could be considered for treatment intensification.</description><subject>692/53/2422</subject><subject>692/699/67/1990/283</subject><subject>692/699/67/2332</subject><subject>Acute lymphoblastic leukemia</subject><subject>Acute lymphocytic leukemia</subject><subject>BCR-ABL protein</subject><subject>Biological and medical sciences</subject><subject>Cancer Research</subject><subject>Cellular signal transduction</subject><subject>Chromosomes</subject><subject>Critical Care Medicine</subject><subject>Cytokines</subject><subject>Down syndrome</subject><subject>Down's syndrome</subject><subject>Fusion protein</subject><subject>Gene expression</subject><subject>Gene Fusion</subject><subject>Genetic aspects</subject><subject>Hematologic and hematopoietic diseases</subject><subject>Hematology</subject><subject>Humans</subject><subject>Impact analysis</subject><subject>Intensive</subject><subject>Internal Medicine</subject><subject>Leukemia</subject><subject>Leukemia in children</subject><subject>Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis</subject><subject>Lymphatic leukemia</subject><subject>Lymphoblastic leukemia in children</subject><subject>Lymphocytes B</subject><subject>Lymphocytic leukemia in children</subject><subject>Medical prognosis</subject><subject>Medical sciences</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Minimal residual disease</subject><subject>Mutation</subject><subject>Oncology</subject><subject>original-article</subject><subject>Overexpression</subject><subject>Patients</subject><subject>Pediatrics</subject><subject>Physiological aspects</subject><subject>Precursor B-Cell Lymphoblastic Leukemia-Lymphoma - genetics</subject><subject>Precursors</subject><subject>Prognosis</subject><subject>Proportional Hazards Models</subject><subject>Receptors, Cytokine - genetics</subject><subject>Receptors, Cytokine - physiology</subject><subject>Receptors, Purinergic P2 - genetics</subject><subject>Recurrence</subject><subject>Risk</subject><subject>Risk Factors</subject><subject>Risk groups</subject><issn>0887-6924</issn><issn>1476-5551</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2012</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><recordid>eNqFkstu1DAUhiMEokNhxxpFQiAWZPA9ybKMKCCNRFXBglXkOPaMW489tR2gb8LjctIZaIsqUBbROf_ncy-KpxjNMaLNG6fHOUGYgIXvFTPMalFxzvH9Yoaapq5ES9hB8SilM4QmUTwsDghhDWMEz4qfJyHEchvDyodkU2nAOiGnX5tqcbo8JqUZkw2-7Mdc-pCv5J0QvulY6R_bqNMVYX2p1tYNUfvyu81rcGQdN3qwMusy2nRevq2Udg6SaTXGBJGkGkFzl5vtOvROpmxVCe2c642Vj4sHRrqkn-z_h8WX43efFx-q5af3HxdHy0rxhuWq5xKLtpcU9Q1GvDeip0pjo3vVDqaRNeOC0WEQ2DSU1K0WvSSGEspbNLnoYfFqFxdmcDHqlLuNTVOd0uswpg5jTAkhGPP_o6jlDaa0bgF9_hd6FsbooZGOCMZrBgWQf1FTLAabE801tZJOd9abkKNUU-ruiCJBGKoRA2p-BwXfANNUwWtjwX_rwcsbD9ZaurxOwY0Ztplug693oIohpahNt412I-MlFNlNJ9jBzrrpBMHCgD_bNzX2sP4_8O-bA-DFHpBJSWei9Mqma04AVZNpOtWOSyD5lY43p3NH4l9HvO9J</recordid><startdate>20121001</startdate><enddate>20121001</enddate><creator>Palmi, C</creator><creator>Vendramini, E</creator><creator>Silvestri, D</creator><creator>Longinotti, G</creator><creator>Frison, D</creator><creator>Cario, G</creator><creator>Shochat, C</creator><creator>Stanulla, M</creator><creator>Rossi, V</creator><creator>Di Meglio, A M</creator><creator>Villa, T</creator><creator>Giarin, E</creator><creator>Fazio, G</creator><creator>Leszl, A</creator><creator>Schrappe, M</creator><creator>Basso, G</creator><creator>Biondi, A</creator><creator>Izraeli, S</creator><creator>Conter, V</creator><creator>Valsecchi, M G</creator><creator>Cazzaniga, G</creator><creator>te Kronnie, G</creator><general>Nature Publishing Group UK</general><general>Nature Publishing Group</general><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>3V.</scope><scope>7QL</scope><scope>7RV</scope><scope>7T5</scope><scope>7T7</scope><scope>7TM</scope><scope>7TO</scope><scope>7U9</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8C1</scope><scope>8FD</scope><scope>8FE</scope><scope>8FH</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BBNVY</scope><scope>BENPR</scope><scope>BHPHI</scope><scope>C1K</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FR3</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>H94</scope><scope>HCIFZ</scope><scope>K9.</scope><scope>KB0</scope><scope>LK8</scope><scope>M0S</scope><scope>M1P</scope><scope>M7N</scope><scope>M7P</scope><scope>NAPCQ</scope><scope>P64</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7X8</scope></search><sort><creationdate>20121001</creationdate><title>Poor prognosis for P2RY8-CRLF2 fusion but not for CRLF2 over-expression in children with intermediate risk B-cell precursor acute lymphoblastic leukemia</title><author>Palmi, C ; Vendramini, E ; Silvestri, D ; Longinotti, G ; Frison, D ; Cario, G ; Shochat, C ; Stanulla, M ; Rossi, V ; Di Meglio, A M ; Villa, T ; Giarin, E ; Fazio, G ; Leszl, A ; Schrappe, M ; Basso, G ; Biondi, A ; Izraeli, S ; Conter, V ; Valsecchi, M G ; Cazzaniga, G ; te Kronnie, G</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c584t-b5a169ba30b8105bf6b3ce1febc9df8a745643dd61f83279e6ba2f3235901f833</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2012</creationdate><topic>692/53/2422</topic><topic>692/699/67/1990/283</topic><topic>692/699/67/2332</topic><topic>Acute lymphoblastic leukemia</topic><topic>Acute lymphocytic leukemia</topic><topic>BCR-ABL protein</topic><topic>Biological and medical sciences</topic><topic>Cancer Research</topic><topic>Cellular signal transduction</topic><topic>Chromosomes</topic><topic>Critical Care Medicine</topic><topic>Cytokines</topic><topic>Down syndrome</topic><topic>Down's syndrome</topic><topic>Fusion protein</topic><topic>Gene expression</topic><topic>Gene Fusion</topic><topic>Genetic aspects</topic><topic>Hematologic and hematopoietic diseases</topic><topic>Hematology</topic><topic>Humans</topic><topic>Impact analysis</topic><topic>Intensive</topic><topic>Internal Medicine</topic><topic>Leukemia</topic><topic>Leukemia in children</topic><topic>Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis</topic><topic>Lymphatic leukemia</topic><topic>Lymphoblastic leukemia in children</topic><topic>Lymphocytes B</topic><topic>Lymphocytic leukemia in children</topic><topic>Medical prognosis</topic><topic>Medical sciences</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Minimal residual disease</topic><topic>Mutation</topic><topic>Oncology</topic><topic>original-article</topic><topic>Overexpression</topic><topic>Patients</topic><topic>Pediatrics</topic><topic>Physiological aspects</topic><topic>Precursor B-Cell Lymphoblastic Leukemia-Lymphoma - genetics</topic><topic>Precursors</topic><topic>Prognosis</topic><topic>Proportional Hazards Models</topic><topic>Receptors, Cytokine - genetics</topic><topic>Receptors, Cytokine - physiology</topic><topic>Receptors, Purinergic P2 - genetics</topic><topic>Recurrence</topic><topic>Risk</topic><topic>Risk Factors</topic><topic>Risk groups</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Palmi, C</creatorcontrib><creatorcontrib>Vendramini, E</creatorcontrib><creatorcontrib>Silvestri, D</creatorcontrib><creatorcontrib>Longinotti, G</creatorcontrib><creatorcontrib>Frison, D</creatorcontrib><creatorcontrib>Cario, G</creatorcontrib><creatorcontrib>Shochat, C</creatorcontrib><creatorcontrib>Stanulla, M</creatorcontrib><creatorcontrib>Rossi, V</creatorcontrib><creatorcontrib>Di Meglio, A M</creatorcontrib><creatorcontrib>Villa, T</creatorcontrib><creatorcontrib>Giarin, E</creatorcontrib><creatorcontrib>Fazio, G</creatorcontrib><creatorcontrib>Leszl, A</creatorcontrib><creatorcontrib>Schrappe, M</creatorcontrib><creatorcontrib>Basso, G</creatorcontrib><creatorcontrib>Biondi, A</creatorcontrib><creatorcontrib>Izraeli, S</creatorcontrib><creatorcontrib>Conter, V</creatorcontrib><creatorcontrib>Valsecchi, M G</creatorcontrib><creatorcontrib>Cazzaniga, G</creatorcontrib><creatorcontrib>te Kronnie, G</creatorcontrib><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>ProQuest Central (Corporate)</collection><collection>Bacteriology Abstracts (Microbiology B)</collection><collection>Nursing & Allied Health Database</collection><collection>Immunology Abstracts</collection><collection>Industrial and Applied Microbiology Abstracts (Microbiology A)</collection><collection>Nucleic Acids Abstracts</collection><collection>Oncogenes and Growth Factors Abstracts</collection><collection>Virology and AIDS Abstracts</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>ProQuest Pharma Collection</collection><collection>Public Health Database</collection><collection>Technology Research Database</collection><collection>ProQuest SciTech Collection</collection><collection>ProQuest Natural Science Collection</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central Essentials</collection><collection>Biological Science Collection</collection><collection>ProQuest Central</collection><collection>Natural Science Collection</collection><collection>Environmental Sciences and Pollution Management</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central Korea</collection><collection>Engineering Research Database</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Central Student</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>SciTech Premium Collection</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Database (Alumni Edition)</collection><collection>ProQuest Biological Science Collection</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Algology Mycology and Protozoology Abstracts (Microbiology C)</collection><collection>Biological Science Database</collection><collection>Nursing & Allied Health Premium</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>MEDLINE - Academic</collection><jtitle>Leukemia</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Palmi, C</au><au>Vendramini, E</au><au>Silvestri, D</au><au>Longinotti, G</au><au>Frison, D</au><au>Cario, G</au><au>Shochat, C</au><au>Stanulla, M</au><au>Rossi, V</au><au>Di Meglio, A M</au><au>Villa, T</au><au>Giarin, E</au><au>Fazio, G</au><au>Leszl, A</au><au>Schrappe, M</au><au>Basso, G</au><au>Biondi, A</au><au>Izraeli, S</au><au>Conter, V</au><au>Valsecchi, M G</au><au>Cazzaniga, G</au><au>te Kronnie, G</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Poor prognosis for P2RY8-CRLF2 fusion but not for CRLF2 over-expression in children with intermediate risk B-cell precursor acute lymphoblastic leukemia</atitle><jtitle>Leukemia</jtitle><stitle>Leukemia</stitle><addtitle>Leukemia</addtitle><date>2012-10-01</date><risdate>2012</risdate><volume>26</volume><issue>10</issue><spage>2245</spage><epage>2253</epage><pages>2245-2253</pages><issn>0887-6924</issn><eissn>1476-5551</eissn><coden>LEUKED</coden><abstract>Pediatric B-cell precursor acute lymphoblastic leukemia (BCP-ALL) has achieved an 80% cure rate as a result of a risk-adapted therapy largely based on minimal residual disease (MRD) monitoring. However, relapse is still the most frequent adverse event, occurring mainly in the patients with intermediate MRD levels (intermediate risk, IR), emphasizing the need for new prognostic markers. We analyzed the prognostic impact of cytokine receptor-like factor 2 (
CRLF2
) over-expression and
P2RY8-CRLF2
fusion in 464 BCP-ALL patients (not affected by Down syndrome and
BCR-ABL
negative) enrolled in the AIEOP-BFM ALL2000 study in Italy. In 22/464 (4.7%) samples, RQ-PCR showed
CRLF2
over-expression (⩾20 times higher than the overall median).
P2RY8-CRLF2
fusion was detected in 22/365 (6%) cases, with 10/22 cases also showing
CRLF2
over-expression.
P2RY8-CRLF2
fusion was the most relevant prognostic factor independent of
CRLF2
over-expression with a threefold increase in risk of relapse. Significantly, the cumulative incidence of relapse of the
P2RY8-CRLF2+
patients in the IR group was high (61.1%±12.9 vs 17.6%±2.6,
P
<0.0001), similar to high-risk patients in AIEOP-BFM ALL2000 study. These results were confirmed in a cohort of patients treated in Germany. In conclusion,
P2RY8-CRLF2
identifies a subset of BCP-ALL patients currently stratified as IR that could be considered for treatment intensification.</abstract><cop>London</cop><pub>Nature Publishing Group UK</pub><pmid>22484421</pmid><doi>10.1038/leu.2012.101</doi><tpages>9</tpages><oa>free_for_read</oa></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0887-6924 |
| ispartof | Leukemia, 2012-10, Vol.26 (10), p.2245-2253 |
| issn | 0887-6924 1476-5551 |
| language | eng |
| recordid | cdi_proquest_miscellaneous_1113222115 |
| source | MEDLINE; Nature; EZB-FREE-00999 freely available EZB journals; Alma/SFX Local Collection |
| subjects | 692/53/2422 692/699/67/1990/283 692/699/67/2332 Acute lymphoblastic leukemia Acute lymphocytic leukemia BCR-ABL protein Biological and medical sciences Cancer Research Cellular signal transduction Chromosomes Critical Care Medicine Cytokines Down syndrome Down's syndrome Fusion protein Gene expression Gene Fusion Genetic aspects Hematologic and hematopoietic diseases Hematology Humans Impact analysis Intensive Internal Medicine Leukemia Leukemia in children Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis Lymphatic leukemia Lymphoblastic leukemia in children Lymphocytes B Lymphocytic leukemia in children Medical prognosis Medical sciences Medicine Medicine & Public Health Minimal residual disease Mutation Oncology original-article Overexpression Patients Pediatrics Physiological aspects Precursor B-Cell Lymphoblastic Leukemia-Lymphoma - genetics Precursors Prognosis Proportional Hazards Models Receptors, Cytokine - genetics Receptors, Cytokine - physiology Receptors, Purinergic P2 - genetics Recurrence Risk Risk Factors Risk groups |
| title | Poor prognosis for P2RY8-CRLF2 fusion but not for CRLF2 over-expression in children with intermediate risk B-cell precursor acute lymphoblastic leukemia |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2024-12-21T14%3A20%3A44IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-gale_proqu&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Poor%20prognosis%20for%20P2RY8-CRLF2%20fusion%20but%20not%20for%20CRLF2%20over-expression%20in%20children%20with%20intermediate%20risk%20B-cell%20precursor%20acute%20lymphoblastic%20leukemia&rft.jtitle=Leukemia&rft.au=Palmi,%20C&rft.date=2012-10-01&rft.volume=26&rft.issue=10&rft.spage=2245&rft.epage=2253&rft.pages=2245-2253&rft.issn=0887-6924&rft.eissn=1476-5551&rft.coden=LEUKED&rft_id=info:doi/10.1038/leu.2012.101&rft_dat=%3Cgale_proqu%3EA306240704%3C/gale_proqu%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=1095492468&rft_id=info:pmid/22484421&rft_galeid=A306240704&rfr_iscdi=true |