Prevalence and distribution of iron overload in patients with transfusion-dependent anemias differs across geographic regions: results from the CORDELIA study
Objectives The randomized comparison of deferasirox to deferoxamine for myocardial iron removal in patients with transfusion‐dependent anemias (CORDELIA) gave the opportunity to assess relative prevalence and body distribution of iron overload in screened patients. Methods Patients aged ≥10 yr with...
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| Veröffentlicht in: | European journal of haematology 2015-09, Vol.95 (3), p.244-253 |
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| creator | Aydinok, Yesim Porter, John B. Piga, Antonio Elalfy, Mohsen El-Beshlawy, Amal Kilinç, Yurdanur Viprakasit, Vip Yesilipek, Akif Habr, Dany Quebe-Fehling, Erhard Pennell, Dudley J. |
| description | Objectives
The randomized comparison of deferasirox to deferoxamine for myocardial iron removal in patients with transfusion‐dependent anemias (CORDELIA) gave the opportunity to assess relative prevalence and body distribution of iron overload in screened patients.
Methods
Patients aged ≥10 yr with transfusion‐dependent anemias from 11 countries were screened. Data were summarized descriptively, overall and across regions.
Results
Among 925 patients (99.1% with β‐thalassemia major; 98.5% receiving prior chelation; mean age 19.2 yr), 36.7% had myocardial iron overload (myocardial T2* ≤20 ms), 12.1% had low left ventricular ejection fraction. Liver iron concentration (LIC) (mean 25.8 mg Fe/g dw) and serum ferritin (median 3702 ng/mL) were high. Fewer patients in the Middle East (ME; 28.5%) had myocardial T2* ≤20 ms vs. patients in the West (45.9%) and Far East (FE, 40.9%). Patients in the West had highest myocardial iron burden, but lowest LIC (26.9% with LIC |
| doi_str_mv | 10.1111/ejh.12487 |
| format | Article |
| fullrecord | <record><control><sourceid>istex_cross</sourceid><recordid>TN_cdi_crossref_primary_10_1111_ejh_12487</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>ark_67375_WNG_PB3W8K16_5</sourcerecordid><originalsourceid>FETCH-LOGICAL-c4687-fab9dddd2994b74752406217fad745e8a6c97e1e42aab690f59760d64f3eb60c3</originalsourceid><addsrcrecordid>eNp1kNFO2zAUhq2JaXSMi73A5Fsu0tqJY8e7Y13XMqoVIRCXlhMft2ZpEtkJrC_Ds2Lo6B3n5ljy9386-hH6SsmYxpnA_WZMU1aID2hEOSEJ4UQeoRGRJE0YY_QYfQ7hnhCSSio-oeM0Z7SghRihpysPD7qGpgKsG4ONC7135dC7tsGtxc6_7AfwdasNdg3udO-g6QN-dP0G9143wQ4h0omBDhoT_6IItk6HKLMWfMC68m0IeA3t2utu4yrsYR0j4Xt8hKGONuvbLe43gKer65-z5cU5Dv1gdl_QR6vrAKf_9wm6_TW7mS6S5Wp-MT1fJhXjhUisLqWJk0rJSsFEnjLCUyqsNoLlUGheSQEUWKp1ySWxuRScGM5sBiUnVXaCzvbe10s9WNV5t9V-pyhRLx2r2LF67Tiy3_ZsN5RbMAfyrdQITPbAo6th975JzX4v3pTJPhHbh3-HhPZ_FReZyNXdn7m6-pHdFZeUqzx7BrkUmNM</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype></control><display><type>article</type><title>Prevalence and distribution of iron overload in patients with transfusion-dependent anemias differs across geographic regions: results from the CORDELIA study</title><source>MEDLINE</source><source>Wiley Online Library Journals Frontfile Complete</source><creator>Aydinok, Yesim ; Porter, John B. ; Piga, Antonio ; Elalfy, Mohsen ; El-Beshlawy, Amal ; Kilinç, Yurdanur ; Viprakasit, Vip ; Yesilipek, Akif ; Habr, Dany ; Quebe-Fehling, Erhard ; Pennell, Dudley J.</creator><creatorcontrib>Aydinok, Yesim ; Porter, John B. ; Piga, Antonio ; Elalfy, Mohsen ; El-Beshlawy, Amal ; Kilinç, Yurdanur ; Viprakasit, Vip ; Yesilipek, Akif ; Habr, Dany ; Quebe-Fehling, Erhard ; Pennell, Dudley J.</creatorcontrib><description>Objectives
The randomized comparison of deferasirox to deferoxamine for myocardial iron removal in patients with transfusion‐dependent anemias (CORDELIA) gave the opportunity to assess relative prevalence and body distribution of iron overload in screened patients.
Methods
Patients aged ≥10 yr with transfusion‐dependent anemias from 11 countries were screened. Data were summarized descriptively, overall and across regions.
Results
Among 925 patients (99.1% with β‐thalassemia major; 98.5% receiving prior chelation; mean age 19.2 yr), 36.7% had myocardial iron overload (myocardial T2* ≤20 ms), 12.1% had low left ventricular ejection fraction. Liver iron concentration (LIC) (mean 25.8 mg Fe/g dw) and serum ferritin (median 3702 ng/mL) were high. Fewer patients in the Middle East (ME; 28.5%) had myocardial T2* ≤20 ms vs. patients in the West (45.9%) and Far East (FE, 40.9%). Patients in the West had highest myocardial iron burden, but lowest LIC (26.9% with LIC <7 mg Fe/g dw) and serum ferritin. Among patients with normal myocardial iron, a higher proportion of patients from the ME and FE had LIC ≥15 than <7 mg Fe/g dw (ME, 56.7% vs. 17.2%; FE, 78.6% vs. 7.8%, respectively), a trend which was less evident in the West (44.6% vs. 33.9%, respectively). Transfusion and chelation practices differed between regions.
Conclusions
Evidence of substantial myocardial and liver iron burden across regions revealed a need for optimization of effective, convenient iron chelation regimens. Significant regional variation exists in myocardial and liver iron loading that are not well explained; improved understanding of factors contributing to differences in body iron distribution may be of clinical benefit.</description><identifier>ISSN: 0902-4441</identifier><identifier>EISSN: 1600-0609</identifier><identifier>DOI: 10.1111/ejh.12487</identifier><identifier>PMID: 25418187</identifier><language>eng</language><publisher>England: Blackwell Publishing Ltd</publisher><subject>Adolescent ; Adult ; Aged ; Aged, 80 and over ; Anemia - complications ; Anemia - therapy ; Child ; Deferoxamine - therapeutic use ; distribution ; Female ; heart ; Humans ; iron ; Iron - metabolism ; Iron Chelating Agents - therapeutic use ; Iron Overload - drug therapy ; Iron Overload - epidemiology ; Iron Overload - etiology ; Iron Overload - metabolism ; liver ; Liver - metabolism ; Liver - pathology ; Male ; Middle Aged ; Myocardium - metabolism ; Myocardium - pathology ; Prevalence ; thalassemia ; Tissue Distribution ; Transfusion Reaction ; Young Adult</subject><ispartof>European journal of haematology, 2015-09, Vol.95 (3), p.244-253</ispartof><rights>2014 The Authors. Published by John Wiley & Sons Ltd.</rights><rights>2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4687-fab9dddd2994b74752406217fad745e8a6c97e1e42aab690f59760d64f3eb60c3</citedby><cites>FETCH-LOGICAL-c4687-fab9dddd2994b74752406217fad745e8a6c97e1e42aab690f59760d64f3eb60c3</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%2Fejh.12487$$EPDF$$P50$$Gwiley$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1111%2Fejh.12487$$EHTML$$P50$$Gwiley$$Hfree_for_read</linktohtml><link.rule.ids>314,777,781,1412,27905,27906,45555,45556</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/25418187$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Aydinok, Yesim</creatorcontrib><creatorcontrib>Porter, John B.</creatorcontrib><creatorcontrib>Piga, Antonio</creatorcontrib><creatorcontrib>Elalfy, Mohsen</creatorcontrib><creatorcontrib>El-Beshlawy, Amal</creatorcontrib><creatorcontrib>Kilinç, Yurdanur</creatorcontrib><creatorcontrib>Viprakasit, Vip</creatorcontrib><creatorcontrib>Yesilipek, Akif</creatorcontrib><creatorcontrib>Habr, Dany</creatorcontrib><creatorcontrib>Quebe-Fehling, Erhard</creatorcontrib><creatorcontrib>Pennell, Dudley J.</creatorcontrib><title>Prevalence and distribution of iron overload in patients with transfusion-dependent anemias differs across geographic regions: results from the CORDELIA study</title><title>European journal of haematology</title><addtitle>Eur J Haematol</addtitle><description>Objectives
The randomized comparison of deferasirox to deferoxamine for myocardial iron removal in patients with transfusion‐dependent anemias (CORDELIA) gave the opportunity to assess relative prevalence and body distribution of iron overload in screened patients.
Methods
Patients aged ≥10 yr with transfusion‐dependent anemias from 11 countries were screened. Data were summarized descriptively, overall and across regions.
Results
Among 925 patients (99.1% with β‐thalassemia major; 98.5% receiving prior chelation; mean age 19.2 yr), 36.7% had myocardial iron overload (myocardial T2* ≤20 ms), 12.1% had low left ventricular ejection fraction. Liver iron concentration (LIC) (mean 25.8 mg Fe/g dw) and serum ferritin (median 3702 ng/mL) were high. Fewer patients in the Middle East (ME; 28.5%) had myocardial T2* ≤20 ms vs. patients in the West (45.9%) and Far East (FE, 40.9%). Patients in the West had highest myocardial iron burden, but lowest LIC (26.9% with LIC <7 mg Fe/g dw) and serum ferritin. Among patients with normal myocardial iron, a higher proportion of patients from the ME and FE had LIC ≥15 than <7 mg Fe/g dw (ME, 56.7% vs. 17.2%; FE, 78.6% vs. 7.8%, respectively), a trend which was less evident in the West (44.6% vs. 33.9%, respectively). Transfusion and chelation practices differed between regions.
Conclusions
Evidence of substantial myocardial and liver iron burden across regions revealed a need for optimization of effective, convenient iron chelation regimens. Significant regional variation exists in myocardial and liver iron loading that are not well explained; improved understanding of factors contributing to differences in body iron distribution may be of clinical benefit.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Aged</subject><subject>Aged, 80 and over</subject><subject>Anemia - complications</subject><subject>Anemia - therapy</subject><subject>Child</subject><subject>Deferoxamine - therapeutic use</subject><subject>distribution</subject><subject>Female</subject><subject>heart</subject><subject>Humans</subject><subject>iron</subject><subject>Iron - metabolism</subject><subject>Iron Chelating Agents - therapeutic use</subject><subject>Iron Overload - drug therapy</subject><subject>Iron Overload - epidemiology</subject><subject>Iron Overload - etiology</subject><subject>Iron Overload - metabolism</subject><subject>liver</subject><subject>Liver - metabolism</subject><subject>Liver - pathology</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Myocardium - metabolism</subject><subject>Myocardium - pathology</subject><subject>Prevalence</subject><subject>thalassemia</subject><subject>Tissue Distribution</subject><subject>Transfusion Reaction</subject><subject>Young Adult</subject><issn>0902-4441</issn><issn>1600-0609</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2015</creationdate><recordtype>article</recordtype><sourceid>24P</sourceid><sourceid>WIN</sourceid><sourceid>EIF</sourceid><recordid>eNp1kNFO2zAUhq2JaXSMi73A5Fsu0tqJY8e7Y13XMqoVIRCXlhMft2ZpEtkJrC_Ds2Lo6B3n5ljy9386-hH6SsmYxpnA_WZMU1aID2hEOSEJ4UQeoRGRJE0YY_QYfQ7hnhCSSio-oeM0Z7SghRihpysPD7qGpgKsG4ONC7135dC7tsGtxc6_7AfwdasNdg3udO-g6QN-dP0G9143wQ4h0omBDhoT_6IItk6HKLMWfMC68m0IeA3t2utu4yrsYR0j4Xt8hKGONuvbLe43gKer65-z5cU5Dv1gdl_QR6vrAKf_9wm6_TW7mS6S5Wp-MT1fJhXjhUisLqWJk0rJSsFEnjLCUyqsNoLlUGheSQEUWKp1ySWxuRScGM5sBiUnVXaCzvbe10s9WNV5t9V-pyhRLx2r2LF67Tiy3_ZsN5RbMAfyrdQITPbAo6th975JzX4v3pTJPhHbh3-HhPZ_FReZyNXdn7m6-pHdFZeUqzx7BrkUmNM</recordid><startdate>201509</startdate><enddate>201509</enddate><creator>Aydinok, Yesim</creator><creator>Porter, John B.</creator><creator>Piga, Antonio</creator><creator>Elalfy, Mohsen</creator><creator>El-Beshlawy, Amal</creator><creator>Kilinç, Yurdanur</creator><creator>Viprakasit, Vip</creator><creator>Yesilipek, Akif</creator><creator>Habr, Dany</creator><creator>Quebe-Fehling, Erhard</creator><creator>Pennell, Dudley J.</creator><general>Blackwell Publishing Ltd</general><scope>BSCLL</scope><scope>24P</scope><scope>WIN</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></search><sort><creationdate>201509</creationdate><title>Prevalence and distribution of iron overload in patients with transfusion-dependent anemias differs across geographic regions: results from the CORDELIA study</title><author>Aydinok, Yesim ; Porter, John B. ; Piga, Antonio ; Elalfy, Mohsen ; El-Beshlawy, Amal ; Kilinç, Yurdanur ; Viprakasit, Vip ; Yesilipek, Akif ; Habr, Dany ; Quebe-Fehling, Erhard ; Pennell, Dudley J.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4687-fab9dddd2994b74752406217fad745e8a6c97e1e42aab690f59760d64f3eb60c3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2015</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Aged</topic><topic>Aged, 80 and over</topic><topic>Anemia - complications</topic><topic>Anemia - therapy</topic><topic>Child</topic><topic>Deferoxamine - therapeutic use</topic><topic>distribution</topic><topic>Female</topic><topic>heart</topic><topic>Humans</topic><topic>iron</topic><topic>Iron - metabolism</topic><topic>Iron Chelating Agents - therapeutic use</topic><topic>Iron Overload - drug therapy</topic><topic>Iron Overload - epidemiology</topic><topic>Iron Overload - etiology</topic><topic>Iron Overload - metabolism</topic><topic>liver</topic><topic>Liver - metabolism</topic><topic>Liver - pathology</topic><topic>Male</topic><topic>Middle Aged</topic><topic>Myocardium - metabolism</topic><topic>Myocardium - pathology</topic><topic>Prevalence</topic><topic>thalassemia</topic><topic>Tissue Distribution</topic><topic>Transfusion Reaction</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Aydinok, Yesim</creatorcontrib><creatorcontrib>Porter, John B.</creatorcontrib><creatorcontrib>Piga, Antonio</creatorcontrib><creatorcontrib>Elalfy, Mohsen</creatorcontrib><creatorcontrib>El-Beshlawy, Amal</creatorcontrib><creatorcontrib>Kilinç, Yurdanur</creatorcontrib><creatorcontrib>Viprakasit, Vip</creatorcontrib><creatorcontrib>Yesilipek, Akif</creatorcontrib><creatorcontrib>Habr, Dany</creatorcontrib><creatorcontrib>Quebe-Fehling, Erhard</creatorcontrib><creatorcontrib>Pennell, Dudley J.</creatorcontrib><collection>Istex</collection><collection>Wiley Online Library Open Access</collection><collection>Wiley Free Content</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><jtitle>European journal of haematology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Aydinok, Yesim</au><au>Porter, John B.</au><au>Piga, Antonio</au><au>Elalfy, Mohsen</au><au>El-Beshlawy, Amal</au><au>Kilinç, Yurdanur</au><au>Viprakasit, Vip</au><au>Yesilipek, Akif</au><au>Habr, Dany</au><au>Quebe-Fehling, Erhard</au><au>Pennell, Dudley J.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Prevalence and distribution of iron overload in patients with transfusion-dependent anemias differs across geographic regions: results from the CORDELIA study</atitle><jtitle>European journal of haematology</jtitle><addtitle>Eur J Haematol</addtitle><date>2015-09</date><risdate>2015</risdate><volume>95</volume><issue>3</issue><spage>244</spage><epage>253</epage><pages>244-253</pages><issn>0902-4441</issn><eissn>1600-0609</eissn><abstract>Objectives
The randomized comparison of deferasirox to deferoxamine for myocardial iron removal in patients with transfusion‐dependent anemias (CORDELIA) gave the opportunity to assess relative prevalence and body distribution of iron overload in screened patients.
Methods
Patients aged ≥10 yr with transfusion‐dependent anemias from 11 countries were screened. Data were summarized descriptively, overall and across regions.
Results
Among 925 patients (99.1% with β‐thalassemia major; 98.5% receiving prior chelation; mean age 19.2 yr), 36.7% had myocardial iron overload (myocardial T2* ≤20 ms), 12.1% had low left ventricular ejection fraction. Liver iron concentration (LIC) (mean 25.8 mg Fe/g dw) and serum ferritin (median 3702 ng/mL) were high. Fewer patients in the Middle East (ME; 28.5%) had myocardial T2* ≤20 ms vs. patients in the West (45.9%) and Far East (FE, 40.9%). Patients in the West had highest myocardial iron burden, but lowest LIC (26.9% with LIC <7 mg Fe/g dw) and serum ferritin. Among patients with normal myocardial iron, a higher proportion of patients from the ME and FE had LIC ≥15 than <7 mg Fe/g dw (ME, 56.7% vs. 17.2%; FE, 78.6% vs. 7.8%, respectively), a trend which was less evident in the West (44.6% vs. 33.9%, respectively). Transfusion and chelation practices differed between regions.
Conclusions
Evidence of substantial myocardial and liver iron burden across regions revealed a need for optimization of effective, convenient iron chelation regimens. Significant regional variation exists in myocardial and liver iron loading that are not well explained; improved understanding of factors contributing to differences in body iron distribution may be of clinical benefit.</abstract><cop>England</cop><pub>Blackwell Publishing Ltd</pub><pmid>25418187</pmid><doi>10.1111/ejh.12487</doi><tpages>10</tpages><oa>free_for_read</oa></addata></record> |
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| identifier | ISSN: 0902-4441 |
| ispartof | European journal of haematology, 2015-09, Vol.95 (3), p.244-253 |
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| language | eng |
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| subjects | Adolescent Adult Aged Aged, 80 and over Anemia - complications Anemia - therapy Child Deferoxamine - therapeutic use distribution Female heart Humans iron Iron - metabolism Iron Chelating Agents - therapeutic use Iron Overload - drug therapy Iron Overload - epidemiology Iron Overload - etiology Iron Overload - metabolism liver Liver - metabolism Liver - pathology Male Middle Aged Myocardium - metabolism Myocardium - pathology Prevalence thalassemia Tissue Distribution Transfusion Reaction Young Adult |
| title | Prevalence and distribution of iron overload in patients with transfusion-dependent anemias differs across geographic regions: results from the CORDELIA study |
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