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A New clinical score for disease activity in Langerhans cell histiocytosis
Objective To develop an objective tool for assessing disease activity in patients with Langerhans cell histiocytosis (LCH). Method Scoring system was developed and applied to a database containing information on 612 patients. Results At diagnosis, the score distribution was highly asymmetrical: the...
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| Published in: | Pediatric Blood & Cancer 2004-12, Vol.43 (7), p.770-776 |
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| Main Authors: | , , , , , , , , , , , |
| Format: | Article |
| Language: | English |
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| cites | cdi_FETCH-LOGICAL-c4860-20aa053c408b59ee4d13edefd6432c56d67e6d535529aeb6c8bcdb2a8e3f3e073 |
| container_end_page | 776 |
| container_issue | 7 |
| container_start_page | 770 |
| container_title | Pediatric Blood & Cancer |
| container_volume | 43 |
| creator | Donadieu, Jean Piguet, C. Bernard, F. Barkaoui, M. Ouache, M. Bertrand, Y. Ibrahim, H. Emile, J.F. Hermine, O. Tazi, A. Genereau, T. Thomas, C. |
| description | Objective
To develop an objective tool for assessing disease activity in patients with Langerhans cell histiocytosis (LCH).
Method
Scoring system was developed and applied to a database containing information on 612 patients.
Results
At diagnosis, the score distribution was highly asymmetrical: the score was between 0 and 2 in 74% of cases, 3–6 in 16%, 7–10 in 3%, and more than 10 in 6%. The 5‐year mortality rates were 1, 4.4, and 43.4%, respectively, among patients with initial scores of 0–2, 3‐6, and >6. Stability or an increase of the score at 6 weeks was highly predictive of death among patients with initial scores above 6, while score stability had no significant impact on vital outcome among patients with low or moderate scores at diagnosis.
Conclusions
This LCH disease activity score provides an objective tool for assessing disease severity, both at diagnosis and during follow‐up and treatment. © 2004 Wiley‐Liss, Inc. |
| doi_str_mv | 10.1002/pbc.20160 |
| format | article |
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To develop an objective tool for assessing disease activity in patients with Langerhans cell histiocytosis (LCH).
Method
Scoring system was developed and applied to a database containing information on 612 patients.
Results
At diagnosis, the score distribution was highly asymmetrical: the score was between 0 and 2 in 74% of cases, 3–6 in 16%, 7–10 in 3%, and more than 10 in 6%. The 5‐year mortality rates were 1, 4.4, and 43.4%, respectively, among patients with initial scores of 0–2, 3‐6, and >6. Stability or an increase of the score at 6 weeks was highly predictive of death among patients with initial scores above 6, while score stability had no significant impact on vital outcome among patients with low or moderate scores at diagnosis.
Conclusions
This LCH disease activity score provides an objective tool for assessing disease severity, both at diagnosis and during follow‐up and treatment. © 2004 Wiley‐Liss, Inc.</description><identifier>ISSN: 1545-5009</identifier><identifier>EISSN: 1545-5017</identifier><identifier>DOI: 10.1002/pbc.20160</identifier><identifier>PMID: 15390280</identifier><language>eng</language><publisher>Hoboken: Wiley Subscription Services, Inc., A Wiley Company</publisher><subject>Antineoplastic Combined Chemotherapy Protocols - therapeutic use ; Biological and medical sciences ; Data Interpretation, Statistical ; Databases, Factual ; General aspects ; Histiocytosis, Langerhans-Cell - diagnosis ; Histiocytosis, Langerhans-Cell - drug therapy ; Histiocytosis, Langerhans-Cell - mortality ; Humans ; Langerhans cell histiocytosis ; Medical sciences ; Predictive Value of Tests ; prognostic factors ; score ; Severity of Illness Index ; Statistical Distributions ; Survival Analysis ; therapeutic evaluation ; Time Factors ; Tumors</subject><ispartof>Pediatric Blood & Cancer, 2004-12, Vol.43 (7), p.770-776</ispartof><rights>Copyright © 2004 Wiley‐Liss, Inc.</rights><rights>2005 INIST-CNRS</rights><rights>2004 Wiley-Liss, Inc.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4860-20aa053c408b59ee4d13edefd6432c56d67e6d535529aeb6c8bcdb2a8e3f3e073</citedby><cites>FETCH-LOGICAL-c4860-20aa053c408b59ee4d13edefd6432c56d67e6d535529aeb6c8bcdb2a8e3f3e073</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=16301826$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/15390280$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Donadieu, Jean</creatorcontrib><creatorcontrib>Piguet, C.</creatorcontrib><creatorcontrib>Bernard, F.</creatorcontrib><creatorcontrib>Barkaoui, M.</creatorcontrib><creatorcontrib>Ouache, M.</creatorcontrib><creatorcontrib>Bertrand, Y.</creatorcontrib><creatorcontrib>Ibrahim, H.</creatorcontrib><creatorcontrib>Emile, J.F.</creatorcontrib><creatorcontrib>Hermine, O.</creatorcontrib><creatorcontrib>Tazi, A.</creatorcontrib><creatorcontrib>Genereau, T.</creatorcontrib><creatorcontrib>Thomas, C.</creatorcontrib><title>A New clinical score for disease activity in Langerhans cell histiocytosis</title><title>Pediatric Blood & Cancer</title><addtitle>Pediatr. Blood Cancer</addtitle><description>Objective
To develop an objective tool for assessing disease activity in patients with Langerhans cell histiocytosis (LCH).
Method
Scoring system was developed and applied to a database containing information on 612 patients.
Results
At diagnosis, the score distribution was highly asymmetrical: the score was between 0 and 2 in 74% of cases, 3–6 in 16%, 7–10 in 3%, and more than 10 in 6%. The 5‐year mortality rates were 1, 4.4, and 43.4%, respectively, among patients with initial scores of 0–2, 3‐6, and >6. Stability or an increase of the score at 6 weeks was highly predictive of death among patients with initial scores above 6, while score stability had no significant impact on vital outcome among patients with low or moderate scores at diagnosis.
Conclusions
This LCH disease activity score provides an objective tool for assessing disease severity, both at diagnosis and during follow‐up and treatment. © 2004 Wiley‐Liss, Inc.</description><subject>Antineoplastic Combined Chemotherapy Protocols - therapeutic use</subject><subject>Biological and medical sciences</subject><subject>Data Interpretation, Statistical</subject><subject>Databases, Factual</subject><subject>General aspects</subject><subject>Histiocytosis, Langerhans-Cell - diagnosis</subject><subject>Histiocytosis, Langerhans-Cell - drug therapy</subject><subject>Histiocytosis, Langerhans-Cell - mortality</subject><subject>Humans</subject><subject>Langerhans cell histiocytosis</subject><subject>Medical sciences</subject><subject>Predictive Value of Tests</subject><subject>prognostic factors</subject><subject>score</subject><subject>Severity of Illness Index</subject><subject>Statistical Distributions</subject><subject>Survival Analysis</subject><subject>therapeutic evaluation</subject><subject>Time Factors</subject><subject>Tumors</subject><issn>1545-5009</issn><issn>1545-5017</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2004</creationdate><recordtype>article</recordtype><recordid>eNqFkE1T2zAQhjVMGaDQQ_8AowvM9GBYSZZsH2kGAplM4AD0qJHldRE4dqp1SvPv6zQpnDo97R6edz8exj4LOBMA8nxR-jMJwsAOOxA61YkGkX1466HYZx-JngfUgM732L7QqgCZwwGbXPAZvnLfhDZ413DyXURed5FXgdARcuf78DP0Kx5aPnXtd4xPriXusWn4U6A-dH7VdxToiO3WriH8tK2H7OHq8n50nUxvxzeji2ni09xAIsE50MqnkJe6QEwrobDCujKpkl6bymRoKq20loXD0vi89FUpXY6qVgiZOmSnm7mL2P1YIvV2Hmh9jmuxW5I1GShTaPNfUIIRwhg1gF82oI8dUcTaLmKYu7iyAuzasB0M2z-GB_Z4O3RZzrF6J7dKB-BkCzgajNbRtT7QO2cUiFyurzvfcK-hwdW_N9q7r6O_q5NNYrCOv94SLr4MP6tM22-zsb2aFNnk-nFmx-o3sx2gnw</recordid><startdate>200412</startdate><enddate>200412</enddate><creator>Donadieu, Jean</creator><creator>Piguet, C.</creator><creator>Bernard, F.</creator><creator>Barkaoui, M.</creator><creator>Ouache, M.</creator><creator>Bertrand, Y.</creator><creator>Ibrahim, H.</creator><creator>Emile, J.F.</creator><creator>Hermine, O.</creator><creator>Tazi, A.</creator><creator>Genereau, T.</creator><creator>Thomas, C.</creator><general>Wiley Subscription Services, Inc., A Wiley Company</general><general>Wiley</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>7T5</scope><scope>H94</scope><scope>7X8</scope></search><sort><creationdate>200412</creationdate><title>A New clinical score for disease activity in Langerhans cell histiocytosis</title><author>Donadieu, Jean ; Piguet, C. ; Bernard, F. ; Barkaoui, M. ; Ouache, M. ; Bertrand, Y. ; Ibrahim, H. ; Emile, J.F. ; Hermine, O. ; Tazi, A. ; Genereau, T. ; Thomas, C.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4860-20aa053c408b59ee4d13edefd6432c56d67e6d535529aeb6c8bcdb2a8e3f3e073</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2004</creationdate><topic>Antineoplastic Combined Chemotherapy Protocols - therapeutic use</topic><topic>Biological and medical sciences</topic><topic>Data Interpretation, Statistical</topic><topic>Databases, Factual</topic><topic>General aspects</topic><topic>Histiocytosis, Langerhans-Cell - diagnosis</topic><topic>Histiocytosis, Langerhans-Cell - drug therapy</topic><topic>Histiocytosis, Langerhans-Cell - mortality</topic><topic>Humans</topic><topic>Langerhans cell histiocytosis</topic><topic>Medical sciences</topic><topic>Predictive Value of Tests</topic><topic>prognostic factors</topic><topic>score</topic><topic>Severity of Illness Index</topic><topic>Statistical Distributions</topic><topic>Survival Analysis</topic><topic>therapeutic evaluation</topic><topic>Time Factors</topic><topic>Tumors</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Donadieu, Jean</creatorcontrib><creatorcontrib>Piguet, C.</creatorcontrib><creatorcontrib>Bernard, F.</creatorcontrib><creatorcontrib>Barkaoui, M.</creatorcontrib><creatorcontrib>Ouache, M.</creatorcontrib><creatorcontrib>Bertrand, Y.</creatorcontrib><creatorcontrib>Ibrahim, H.</creatorcontrib><creatorcontrib>Emile, J.F.</creatorcontrib><creatorcontrib>Hermine, O.</creatorcontrib><creatorcontrib>Tazi, A.</creatorcontrib><creatorcontrib>Genereau, T.</creatorcontrib><creatorcontrib>Thomas, C.</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>Immunology Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>Pediatric Blood & Cancer</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Donadieu, Jean</au><au>Piguet, C.</au><au>Bernard, F.</au><au>Barkaoui, M.</au><au>Ouache, M.</au><au>Bertrand, Y.</au><au>Ibrahim, H.</au><au>Emile, J.F.</au><au>Hermine, O.</au><au>Tazi, A.</au><au>Genereau, T.</au><au>Thomas, C.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>A New clinical score for disease activity in Langerhans cell histiocytosis</atitle><jtitle>Pediatric Blood & Cancer</jtitle><addtitle>Pediatr. Blood Cancer</addtitle><date>2004-12</date><risdate>2004</risdate><volume>43</volume><issue>7</issue><spage>770</spage><epage>776</epage><pages>770-776</pages><issn>1545-5009</issn><eissn>1545-5017</eissn><abstract>Objective
To develop an objective tool for assessing disease activity in patients with Langerhans cell histiocytosis (LCH).
Method
Scoring system was developed and applied to a database containing information on 612 patients.
Results
At diagnosis, the score distribution was highly asymmetrical: the score was between 0 and 2 in 74% of cases, 3–6 in 16%, 7–10 in 3%, and more than 10 in 6%. The 5‐year mortality rates were 1, 4.4, and 43.4%, respectively, among patients with initial scores of 0–2, 3‐6, and >6. Stability or an increase of the score at 6 weeks was highly predictive of death among patients with initial scores above 6, while score stability had no significant impact on vital outcome among patients with low or moderate scores at diagnosis.
Conclusions
This LCH disease activity score provides an objective tool for assessing disease severity, both at diagnosis and during follow‐up and treatment. © 2004 Wiley‐Liss, Inc.</abstract><cop>Hoboken</cop><pub>Wiley Subscription Services, Inc., A Wiley Company</pub><pmid>15390280</pmid><doi>10.1002/pbc.20160</doi><tpages>7</tpages></addata></record> |
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| ispartof | Pediatric Blood & Cancer, 2004-12, Vol.43 (7), p.770-776 |
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| subjects | Antineoplastic Combined Chemotherapy Protocols - therapeutic use Biological and medical sciences Data Interpretation, Statistical Databases, Factual General aspects Histiocytosis, Langerhans-Cell - diagnosis Histiocytosis, Langerhans-Cell - drug therapy Histiocytosis, Langerhans-Cell - mortality Humans Langerhans cell histiocytosis Medical sciences Predictive Value of Tests prognostic factors score Severity of Illness Index Statistical Distributions Survival Analysis therapeutic evaluation Time Factors Tumors |
| title | A New clinical score for disease activity in Langerhans cell histiocytosis |
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