Diffuse large B‐cell lymphoma (Richter syndrome) in patients with chronic lymphocytic leukaemia (CLL): a cohort study of newly diagnosed patients
Summary Nearly all information about patients with chronic lymphocytic leukaemia (CLL) who develop diffuse large B‐cell lymphoma [Richter syndrome (RS)] is derived from retrospective case series or patients treated on clinical trials. We used the Mayo Clinic CLL Database to identify patients with ne...
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Veröffentlicht in: | British journal of haematology 2013-09, Vol.162 (6), p.774-782 |
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creator | Parikh, Sameer A. Rabe, Kari G. Call, Timothy G. Zent, Clive S. Habermann, Thomas M. Ding, Wei Leis, Jose F. Schwager, Susan M. Hanson, Curtis A. Macon, William R. Kay, Neil E. Slager, Susan L. Shanafelt, Tait D. |
description | Summary
Nearly all information about patients with chronic lymphocytic leukaemia (CLL) who develop diffuse large B‐cell lymphoma [Richter syndrome (RS)] is derived from retrospective case series or patients treated on clinical trials. We used the Mayo Clinic CLL Database to identify patients with newly diagnosed CLL between January 2000 and July 2011. Individuals who developed biopsy‐proven RS during follow‐up were identified. After a median follow‐up of 4 years, 37/1641 (2·3%) CLL patients developed RS. The rate of RS was approximately 0·5%/year. Risk of RS was associated with advanced Rai stage at diagnosis (P |
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Nearly all information about patients with chronic lymphocytic leukaemia (CLL) who develop diffuse large B‐cell lymphoma [Richter syndrome (RS)] is derived from retrospective case series or patients treated on clinical trials. We used the Mayo Clinic CLL Database to identify patients with newly diagnosed CLL between January 2000 and July 2011. Individuals who developed biopsy‐proven RS during follow‐up were identified. After a median follow‐up of 4 years, 37/1641 (2·3%) CLL patients developed RS. The rate of RS was approximately 0·5%/year. Risk of RS was associated with advanced Rai stage at diagnosis (P < 0·001), high‐risk genetic abnormalitites on fluorescence in situ hybridization (P < 0·0001), unmutated IGHV (P = 0·003), and expression of ZAP70 (P = 0·02) and CD38 (P = 0·001). The rate of RS doubled in patients after treatment for CLL (1%/year). Stereotyped B‐cell receptors (odds‐ratio = 4·2; P = 0·01) but not IGHV4‐39 family usage was associated with increased risk of RS. Treatment with combination of purine analogues and alkylating agents increased the risk of RS three‐fold (odds‐ratio = 3·26, P = 0·0003). Median survival after RS diagnosis was 2·1 years. The RS prognosis score stratified patients into three risk groups with median survivals of 0·5 years, 2·1 years and not reached. Both underlying characteristics of the CLL clone and subsequent CLL therapy influence the risk of RS. Survival after RS remains poor and new therapies are needed.</description><identifier>ISSN: 0007-1048</identifier><identifier>EISSN: 1365-2141</identifier><identifier>DOI: 10.1111/bjh.12458</identifier><identifier>PMID: 23841899</identifier><identifier>CODEN: BJHEAL</identifier><language>eng</language><publisher>Oxford: Blackwell</publisher><subject>Adult ; Aged ; Aged, 80 and over ; aggressive lymphoma ; Biological and medical sciences ; Cohort Studies ; Disease Progression ; Female ; Hematologic and hematopoietic diseases ; Humans ; Leukemia, Lymphocytic, Chronic, B-Cell - complications ; Leukemia, Lymphocytic, Chronic, B-Cell - drug therapy ; Leukemia, Lymphocytic, Chronic, B-Cell - surgery ; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis ; Lymphoma, Large B-Cell, Diffuse - complications ; Lymphoma, Large B-Cell, Diffuse - drug therapy ; Lymphoma, Large B-Cell, Diffuse - surgery ; Male ; Medical sciences ; Middle Aged ; Prognosis ; purine analogues ; Richter syndrome survival score ; stem cell transplantation ; Stem Cell Transplantation - methods ; Survival Rate ; transformation ; Tumors ; Young Adult</subject><ispartof>British journal of haematology, 2013-09, Vol.162 (6), p.774-782</ispartof><rights>2013 John Wiley & Sons Ltd</rights><rights>2014 INIST-CNRS</rights><rights>2013 John Wiley & Sons Ltd.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4788-802a2b1e57be577ebf6ed7a297deb464f645593ad42d5ab9c91690aa82c0aed13</citedby><cites>FETCH-LOGICAL-c4788-802a2b1e57be577ebf6ed7a297deb464f645593ad42d5ab9c91690aa82c0aed13</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1111%2Fbjh.12458$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1111%2Fbjh.12458$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>230,314,780,784,885,1417,1433,27924,27925,45574,45575,46409,46833</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=27720650$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/23841899$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Parikh, Sameer A.</creatorcontrib><creatorcontrib>Rabe, Kari G.</creatorcontrib><creatorcontrib>Call, Timothy G.</creatorcontrib><creatorcontrib>Zent, Clive S.</creatorcontrib><creatorcontrib>Habermann, Thomas M.</creatorcontrib><creatorcontrib>Ding, Wei</creatorcontrib><creatorcontrib>Leis, Jose F.</creatorcontrib><creatorcontrib>Schwager, Susan M.</creatorcontrib><creatorcontrib>Hanson, Curtis A.</creatorcontrib><creatorcontrib>Macon, William R.</creatorcontrib><creatorcontrib>Kay, Neil E.</creatorcontrib><creatorcontrib>Slager, Susan L.</creatorcontrib><creatorcontrib>Shanafelt, Tait D.</creatorcontrib><title>Diffuse large B‐cell lymphoma (Richter syndrome) in patients with chronic lymphocytic leukaemia (CLL): a cohort study of newly diagnosed patients</title><title>British journal of haematology</title><addtitle>Br J Haematol</addtitle><description>Summary
Nearly all information about patients with chronic lymphocytic leukaemia (CLL) who develop diffuse large B‐cell lymphoma [Richter syndrome (RS)] is derived from retrospective case series or patients treated on clinical trials. We used the Mayo Clinic CLL Database to identify patients with newly diagnosed CLL between January 2000 and July 2011. Individuals who developed biopsy‐proven RS during follow‐up were identified. After a median follow‐up of 4 years, 37/1641 (2·3%) CLL patients developed RS. The rate of RS was approximately 0·5%/year. Risk of RS was associated with advanced Rai stage at diagnosis (P < 0·001), high‐risk genetic abnormalitites on fluorescence in situ hybridization (P < 0·0001), unmutated IGHV (P = 0·003), and expression of ZAP70 (P = 0·02) and CD38 (P = 0·001). The rate of RS doubled in patients after treatment for CLL (1%/year). Stereotyped B‐cell receptors (odds‐ratio = 4·2; P = 0·01) but not IGHV4‐39 family usage was associated with increased risk of RS. Treatment with combination of purine analogues and alkylating agents increased the risk of RS three‐fold (odds‐ratio = 3·26, P = 0·0003). Median survival after RS diagnosis was 2·1 years. The RS prognosis score stratified patients into three risk groups with median survivals of 0·5 years, 2·1 years and not reached. Both underlying characteristics of the CLL clone and subsequent CLL therapy influence the risk of RS. Survival after RS remains poor and new therapies are needed.</description><subject>Adult</subject><subject>Aged</subject><subject>Aged, 80 and over</subject><subject>aggressive lymphoma</subject><subject>Biological and medical sciences</subject><subject>Cohort Studies</subject><subject>Disease Progression</subject><subject>Female</subject><subject>Hematologic and hematopoietic diseases</subject><subject>Humans</subject><subject>Leukemia, Lymphocytic, Chronic, B-Cell - complications</subject><subject>Leukemia, Lymphocytic, Chronic, B-Cell - drug therapy</subject><subject>Leukemia, Lymphocytic, Chronic, B-Cell - surgery</subject><subject>Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis</subject><subject>Lymphoma, Large B-Cell, Diffuse - complications</subject><subject>Lymphoma, Large B-Cell, Diffuse - drug therapy</subject><subject>Lymphoma, Large B-Cell, Diffuse - surgery</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Middle Aged</subject><subject>Prognosis</subject><subject>purine analogues</subject><subject>Richter syndrome survival score</subject><subject>stem cell transplantation</subject><subject>Stem Cell Transplantation - methods</subject><subject>Survival Rate</subject><subject>transformation</subject><subject>Tumors</subject><subject>Young Adult</subject><issn>0007-1048</issn><issn>1365-2141</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2013</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNkdGK1DAUhoso7rh64QtIboSdi9lN0rRpvRDcWXWVAUH0Opwmp9OsbTMmrUPvfIQF39AnMeOMo14IBkIO5Dv_n5w_SR4zes7iuqhumnPGRVbcSWYszbMFZ4LdTWaUUrlgVBQnyYMQbihlKc3Y_eSEp4VgRVnOkm9Xtq7HgKQFv0Zy-f3rrca2Je3UbRrXATl7b3UzoCdh6o13Hc6J7ckGBov9EMjWDg3RjXe91YcmPQ27GsdPgJ2NCsvVav6MANGucX4gYRjNRFxNety2EzEW1r0LaI6iD5N7NbQBHx3O0-Tjq5cflteL1bvXb5YvVgstZFEsCsqBVwwzWcUtsapzNBJ4KQ1WIhd1LrKsTMEIbjKoSl2yvKQABdcU0LD0NHm-192MVYdGR28Prdp424GflAOr_r7pbaPW7osStCwKkUWBs4OAd59HDIPqbNiND3p0Y1BMpCVnUsj_QXmZpyyneUTne1R7F4LH-vgiRtUubxXzVj_zjuyTP79wJH8FHIGnBwCChrb20GsbfnNScppnNHIXe25rW5z-7agu317vrX8AcIDFQQ</recordid><startdate>201309</startdate><enddate>201309</enddate><creator>Parikh, Sameer A.</creator><creator>Rabe, Kari G.</creator><creator>Call, Timothy G.</creator><creator>Zent, Clive S.</creator><creator>Habermann, Thomas M.</creator><creator>Ding, Wei</creator><creator>Leis, Jose F.</creator><creator>Schwager, Susan M.</creator><creator>Hanson, Curtis A.</creator><creator>Macon, William R.</creator><creator>Kay, Neil E.</creator><creator>Slager, Susan L.</creator><creator>Shanafelt, Tait D.</creator><general>Blackwell</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>7X8</scope><scope>7T5</scope><scope>H94</scope><scope>5PM</scope></search><sort><creationdate>201309</creationdate><title>Diffuse large B‐cell lymphoma (Richter syndrome) in patients with chronic lymphocytic leukaemia (CLL): a cohort study of newly diagnosed patients</title><author>Parikh, Sameer A. ; Rabe, Kari G. ; Call, Timothy G. ; Zent, Clive S. ; Habermann, Thomas M. ; Ding, Wei ; Leis, Jose F. ; Schwager, Susan M. ; Hanson, Curtis A. ; Macon, William R. ; Kay, Neil E. ; Slager, Susan L. ; Shanafelt, Tait D.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4788-802a2b1e57be577ebf6ed7a297deb464f645593ad42d5ab9c91690aa82c0aed13</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2013</creationdate><topic>Adult</topic><topic>Aged</topic><topic>Aged, 80 and over</topic><topic>aggressive lymphoma</topic><topic>Biological and medical sciences</topic><topic>Cohort Studies</topic><topic>Disease Progression</topic><topic>Female</topic><topic>Hematologic and hematopoietic diseases</topic><topic>Humans</topic><topic>Leukemia, Lymphocytic, Chronic, B-Cell - complications</topic><topic>Leukemia, Lymphocytic, Chronic, B-Cell - drug therapy</topic><topic>Leukemia, Lymphocytic, Chronic, B-Cell - surgery</topic><topic>Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis</topic><topic>Lymphoma, Large B-Cell, Diffuse - complications</topic><topic>Lymphoma, Large B-Cell, Diffuse - drug therapy</topic><topic>Lymphoma, Large B-Cell, Diffuse - surgery</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Middle Aged</topic><topic>Prognosis</topic><topic>purine analogues</topic><topic>Richter syndrome survival score</topic><topic>stem cell transplantation</topic><topic>Stem Cell Transplantation - methods</topic><topic>Survival Rate</topic><topic>transformation</topic><topic>Tumors</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Parikh, Sameer A.</creatorcontrib><creatorcontrib>Rabe, Kari G.</creatorcontrib><creatorcontrib>Call, Timothy G.</creatorcontrib><creatorcontrib>Zent, Clive S.</creatorcontrib><creatorcontrib>Habermann, Thomas M.</creatorcontrib><creatorcontrib>Ding, Wei</creatorcontrib><creatorcontrib>Leis, Jose F.</creatorcontrib><creatorcontrib>Schwager, Susan M.</creatorcontrib><creatorcontrib>Hanson, Curtis A.</creatorcontrib><creatorcontrib>Macon, William R.</creatorcontrib><creatorcontrib>Kay, Neil E.</creatorcontrib><creatorcontrib>Slager, Susan L.</creatorcontrib><creatorcontrib>Shanafelt, Tait D.</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>MEDLINE - Academic</collection><collection>Immunology Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>British journal of haematology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Parikh, Sameer A.</au><au>Rabe, Kari G.</au><au>Call, Timothy G.</au><au>Zent, Clive S.</au><au>Habermann, Thomas M.</au><au>Ding, Wei</au><au>Leis, Jose F.</au><au>Schwager, Susan M.</au><au>Hanson, Curtis A.</au><au>Macon, William R.</au><au>Kay, Neil E.</au><au>Slager, Susan L.</au><au>Shanafelt, Tait D.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Diffuse large B‐cell lymphoma (Richter syndrome) in patients with chronic lymphocytic leukaemia (CLL): a cohort study of newly diagnosed patients</atitle><jtitle>British journal of haematology</jtitle><addtitle>Br J Haematol</addtitle><date>2013-09</date><risdate>2013</risdate><volume>162</volume><issue>6</issue><spage>774</spage><epage>782</epage><pages>774-782</pages><issn>0007-1048</issn><eissn>1365-2141</eissn><coden>BJHEAL</coden><abstract>Summary
Nearly all information about patients with chronic lymphocytic leukaemia (CLL) who develop diffuse large B‐cell lymphoma [Richter syndrome (RS)] is derived from retrospective case series or patients treated on clinical trials. We used the Mayo Clinic CLL Database to identify patients with newly diagnosed CLL between January 2000 and July 2011. Individuals who developed biopsy‐proven RS during follow‐up were identified. After a median follow‐up of 4 years, 37/1641 (2·3%) CLL patients developed RS. The rate of RS was approximately 0·5%/year. Risk of RS was associated with advanced Rai stage at diagnosis (P < 0·001), high‐risk genetic abnormalitites on fluorescence in situ hybridization (P < 0·0001), unmutated IGHV (P = 0·003), and expression of ZAP70 (P = 0·02) and CD38 (P = 0·001). The rate of RS doubled in patients after treatment for CLL (1%/year). Stereotyped B‐cell receptors (odds‐ratio = 4·2; P = 0·01) but not IGHV4‐39 family usage was associated with increased risk of RS. Treatment with combination of purine analogues and alkylating agents increased the risk of RS three‐fold (odds‐ratio = 3·26, P = 0·0003). Median survival after RS diagnosis was 2·1 years. The RS prognosis score stratified patients into three risk groups with median survivals of 0·5 years, 2·1 years and not reached. Both underlying characteristics of the CLL clone and subsequent CLL therapy influence the risk of RS. Survival after RS remains poor and new therapies are needed.</abstract><cop>Oxford</cop><pub>Blackwell</pub><pmid>23841899</pmid><doi>10.1111/bjh.12458</doi><tpages>9</tpages><oa>free_for_read</oa></addata></record> |
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subjects | Adult Aged Aged, 80 and over aggressive lymphoma Biological and medical sciences Cohort Studies Disease Progression Female Hematologic and hematopoietic diseases Humans Leukemia, Lymphocytic, Chronic, B-Cell - complications Leukemia, Lymphocytic, Chronic, B-Cell - drug therapy Leukemia, Lymphocytic, Chronic, B-Cell - surgery Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis Lymphoma, Large B-Cell, Diffuse - complications Lymphoma, Large B-Cell, Diffuse - drug therapy Lymphoma, Large B-Cell, Diffuse - surgery Male Medical sciences Middle Aged Prognosis purine analogues Richter syndrome survival score stem cell transplantation Stem Cell Transplantation - methods Survival Rate transformation Tumors Young Adult |
title | Diffuse large B‐cell lymphoma (Richter syndrome) in patients with chronic lymphocytic leukaemia (CLL): a cohort study of newly diagnosed patients |
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