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Comparative study on baseline clinical characteristics of Asian versus non-Asian patients with paroxysmal nocturnal hemoglobinuria
A difference in clinical manifestations of paroxysmal nocturnal hemoglobinuria (PNH) among different races has been suggested. The aim of this study was to clarify whether the clinical characteristics of patients with PNH in the International PNH Registry differ by ethnic background. Patients, who w...
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Published in: | International journal of hematology 2019-10, Vol.110 (4), p.411-418 |
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creator | Sakurai, Masatoshi Jang, Jun Ho Chou, Wen-Chien Kim, Jin Seok Wilson, Amanda Nishimura, Jun-ichi Chiou, Tzeon-Jye Kanakura, Yuzuru Lee, Jong Wook Okamoto, Shinichiro |
description | A difference in clinical manifestations of paroxysmal nocturnal hemoglobinuria (PNH) among different races has been suggested. The aim of this study was to clarify whether the clinical characteristics of patients with PNH in the International PNH Registry differ by ethnic background. Patients, who were eculizumab naïve at baseline and had ≥ 1% PNH clone size, were eligible for this analysis. Totally, 1793 patients were enrolled and divided into two cohorts, Asian (
N
= 246) and non-Asian (
N
= 1547). The Asian cohort was further divided into Asians in Asia cohort (
N
= 202) and Asians in non-Asia cohort (
N
= 44), based on geographical region. The Asian cohort had significantly higher PNH clone size in granulocytes, higher lactate dehydrogenase levels, and lower hemoglobin levels. However, the frequencies of symptoms including abdominal pain, backache, easy bleeding, fatigue and headache at baseline were significantly lower in the Asian cohort. The proportion of patients with a history of thromboembolism (TE) was significantly lower in the Asian than in the non-Asian cohort (3.6% vs. 8.9%,
P |
doi_str_mv | 10.1007/s12185-019-02699-7 |
format | article |
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N
= 246) and non-Asian (
N
= 1547). The Asian cohort was further divided into Asians in Asia cohort (
N
= 202) and Asians in non-Asia cohort (
N
= 44), based on geographical region. The Asian cohort had significantly higher PNH clone size in granulocytes, higher lactate dehydrogenase levels, and lower hemoglobin levels. However, the frequencies of symptoms including abdominal pain, backache, easy bleeding, fatigue and headache at baseline were significantly lower in the Asian cohort. The proportion of patients with a history of thromboembolism (TE) was significantly lower in the Asian than in the non-Asian cohort (3.6% vs. 8.9%,
P
< 0.01); however, there was no difference between Asians in Asia and Asians in non-Asia (3.3% vs. 4.9%,
P
= 0.61). These findings suggested that genetic factors may play a stronger role in developing TE than lifestyle factors in PNH patients.</description><identifier>ISSN: 0925-5710</identifier><identifier>EISSN: 1865-3774</identifier><identifier>DOI: 10.1007/s12185-019-02699-7</identifier><identifier>PMID: 31278635</identifier><language>eng</language><publisher>Tokyo: Springer Japan</publisher><subject>Abdominal Pain - epidemiology ; Abdominal Pain - etiology ; Adult ; Asian Continental Ancestry Group ; Asian people ; Back Pain - epidemiology ; Back Pain - etiology ; Bleeding ; Cloning ; Cohort Studies ; Comparative studies ; Continental Population Groups ; Fatigue - epidemiology ; Fatigue - etiology ; Female ; Genetic factors ; Granulocytes - pathology ; Headache ; Headache - epidemiology ; Headache - etiology ; Hematology ; Hemoglobin ; Hemoglobins ; Hemoglobinuria, Paroxysmal - blood ; Hemoglobinuria, Paroxysmal - complications ; Hemoglobinuria, Paroxysmal - genetics ; Hemoglobinuria, Paroxysmal - physiopathology ; Hemorrhage - epidemiology ; Hemorrhage - etiology ; Humans ; L-Lactate dehydrogenase ; L-Lactate Dehydrogenase - metabolism ; Lactate dehydrogenase ; Lactic acid ; Leukocytes (granulocytic) ; Male ; Medicine ; Medicine & Public Health ; Middle Aged ; Oncology ; Original Article ; Pain ; Paroxysmal nocturnal hemoglobinuria ; Registries ; Signs and symptoms ; Thromboembolism ; Thromboembolism - epidemiology ; Thromboembolism - etiology</subject><ispartof>International journal of hematology, 2019-10, Vol.110 (4), p.411-418</ispartof><rights>Japanese Society of Hematology 2019</rights><rights>International Journal of Hematology is a copyright of Springer, (2019). All Rights Reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c399t-7b904317d3f45bc61b495ac78643dd4d89cd4313472ec07aaca721178669f4963</citedby><cites>FETCH-LOGICAL-c399t-7b904317d3f45bc61b495ac78643dd4d89cd4313472ec07aaca721178669f4963</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/31278635$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Sakurai, Masatoshi</creatorcontrib><creatorcontrib>Jang, Jun Ho</creatorcontrib><creatorcontrib>Chou, Wen-Chien</creatorcontrib><creatorcontrib>Kim, Jin Seok</creatorcontrib><creatorcontrib>Wilson, Amanda</creatorcontrib><creatorcontrib>Nishimura, Jun-ichi</creatorcontrib><creatorcontrib>Chiou, Tzeon-Jye</creatorcontrib><creatorcontrib>Kanakura, Yuzuru</creatorcontrib><creatorcontrib>Lee, Jong Wook</creatorcontrib><creatorcontrib>Okamoto, Shinichiro</creatorcontrib><title>Comparative study on baseline clinical characteristics of Asian versus non-Asian patients with paroxysmal nocturnal hemoglobinuria</title><title>International journal of hematology</title><addtitle>Int J Hematol</addtitle><addtitle>Int J Hematol</addtitle><description>A difference in clinical manifestations of paroxysmal nocturnal hemoglobinuria (PNH) among different races has been suggested. The aim of this study was to clarify whether the clinical characteristics of patients with PNH in the International PNH Registry differ by ethnic background. Patients, who were eculizumab naïve at baseline and had ≥ 1% PNH clone size, were eligible for this analysis. Totally, 1793 patients were enrolled and divided into two cohorts, Asian (
N
= 246) and non-Asian (
N
= 1547). The Asian cohort was further divided into Asians in Asia cohort (
N
= 202) and Asians in non-Asia cohort (
N
= 44), based on geographical region. The Asian cohort had significantly higher PNH clone size in granulocytes, higher lactate dehydrogenase levels, and lower hemoglobin levels. However, the frequencies of symptoms including abdominal pain, backache, easy bleeding, fatigue and headache at baseline were significantly lower in the Asian cohort. The proportion of patients with a history of thromboembolism (TE) was significantly lower in the Asian than in the non-Asian cohort (3.6% vs. 8.9%,
P
< 0.01); however, there was no difference between Asians in Asia and Asians in non-Asia (3.3% vs. 4.9%,
P
= 0.61). These findings suggested that genetic factors may play a stronger role in developing TE than lifestyle factors in PNH patients.</description><subject>Abdominal Pain - epidemiology</subject><subject>Abdominal Pain - etiology</subject><subject>Adult</subject><subject>Asian Continental Ancestry Group</subject><subject>Asian people</subject><subject>Back Pain - epidemiology</subject><subject>Back Pain - etiology</subject><subject>Bleeding</subject><subject>Cloning</subject><subject>Cohort Studies</subject><subject>Comparative studies</subject><subject>Continental Population Groups</subject><subject>Fatigue - epidemiology</subject><subject>Fatigue - etiology</subject><subject>Female</subject><subject>Genetic factors</subject><subject>Granulocytes - pathology</subject><subject>Headache</subject><subject>Headache - epidemiology</subject><subject>Headache - etiology</subject><subject>Hematology</subject><subject>Hemoglobin</subject><subject>Hemoglobins</subject><subject>Hemoglobinuria, Paroxysmal - blood</subject><subject>Hemoglobinuria, Paroxysmal - complications</subject><subject>Hemoglobinuria, Paroxysmal - genetics</subject><subject>Hemoglobinuria, Paroxysmal - physiopathology</subject><subject>Hemorrhage - epidemiology</subject><subject>Hemorrhage - etiology</subject><subject>Humans</subject><subject>L-Lactate dehydrogenase</subject><subject>L-Lactate Dehydrogenase - metabolism</subject><subject>Lactate dehydrogenase</subject><subject>Lactic acid</subject><subject>Leukocytes (granulocytic)</subject><subject>Male</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Middle Aged</subject><subject>Oncology</subject><subject>Original Article</subject><subject>Pain</subject><subject>Paroxysmal nocturnal hemoglobinuria</subject><subject>Registries</subject><subject>Signs and symptoms</subject><subject>Thromboembolism</subject><subject>Thromboembolism - epidemiology</subject><subject>Thromboembolism - etiology</subject><issn>0925-5710</issn><issn>1865-3774</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2019</creationdate><recordtype>article</recordtype><recordid>eNp9kUtv1DAUhS1ERafT_gEWyBIbNgY_43hZjXhUqsSGriPHcTquEnvwdQqz5ZdjJgUkFmx8bZ_vHj8OQi8Zfcso1e-AcdYqQpkhlDfGEP0MbVjbKCK0ls_RhhquiNKMnqMLgAdKmaZSv0DngnHdNkJt0I9dmg822xIePYayDEecIu4t-ClEj10dg7MTdvsKueJzgBIc4DTiawg24kefYQEcUyTrxqF6-VgAfwtlX1c5fT_CXC1icmXJsc72fk73U-pDXHKwl-hstBP4q6e6RXcf3n_ZfSK3nz_e7K5viRPGFKJ7Q6VgehCjVL1rWC-Nsq6-Q4phkENr3FB1ITX3jmprndWcsao3ZpSmEVv0ZvU95PR18VC6OYDz02SjTwt0nCtRv0XUskWv_0Ef0unqJ4orRdsTxVfK5QSQ_dgdcphtPnaMdr8S6taEuppQd0qo07Xp1ZP10s9--NPyO5IKiBWAKsV7n_-e_R_bn-81neE</recordid><startdate>20191001</startdate><enddate>20191001</enddate><creator>Sakurai, Masatoshi</creator><creator>Jang, Jun Ho</creator><creator>Chou, Wen-Chien</creator><creator>Kim, Jin Seok</creator><creator>Wilson, Amanda</creator><creator>Nishimura, Jun-ichi</creator><creator>Chiou, Tzeon-Jye</creator><creator>Kanakura, Yuzuru</creator><creator>Lee, Jong Wook</creator><creator>Okamoto, Shinichiro</creator><general>Springer Japan</general><general>Springer Nature B.V</general><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>7RV</scope><scope>7T5</scope><scope>7T7</scope><scope>7TM</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8FD</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>BENPR</scope><scope>C1K</scope><scope>CCPQU</scope><scope>FR3</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>H94</scope><scope>K9.</scope><scope>KB0</scope><scope>M0S</scope><scope>M1P</scope><scope>NAPCQ</scope><scope>P64</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>7X8</scope></search><sort><creationdate>20191001</creationdate><title>Comparative study on baseline clinical characteristics of Asian versus non-Asian patients with paroxysmal nocturnal hemoglobinuria</title><author>Sakurai, Masatoshi ; Jang, Jun Ho ; Chou, Wen-Chien ; Kim, Jin Seok ; Wilson, Amanda ; Nishimura, Jun-ichi ; Chiou, Tzeon-Jye ; Kanakura, Yuzuru ; Lee, Jong Wook ; Okamoto, Shinichiro</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c399t-7b904317d3f45bc61b495ac78643dd4d89cd4313472ec07aaca721178669f4963</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2019</creationdate><topic>Abdominal Pain - epidemiology</topic><topic>Abdominal Pain - etiology</topic><topic>Adult</topic><topic>Asian Continental Ancestry Group</topic><topic>Asian people</topic><topic>Back Pain - epidemiology</topic><topic>Back Pain - etiology</topic><topic>Bleeding</topic><topic>Cloning</topic><topic>Cohort Studies</topic><topic>Comparative studies</topic><topic>Continental Population Groups</topic><topic>Fatigue - epidemiology</topic><topic>Fatigue - etiology</topic><topic>Female</topic><topic>Genetic factors</topic><topic>Granulocytes - pathology</topic><topic>Headache</topic><topic>Headache - epidemiology</topic><topic>Headache - etiology</topic><topic>Hematology</topic><topic>Hemoglobin</topic><topic>Hemoglobins</topic><topic>Hemoglobinuria, Paroxysmal - blood</topic><topic>Hemoglobinuria, Paroxysmal - complications</topic><topic>Hemoglobinuria, Paroxysmal - genetics</topic><topic>Hemoglobinuria, Paroxysmal - physiopathology</topic><topic>Hemorrhage - epidemiology</topic><topic>Hemorrhage - etiology</topic><topic>Humans</topic><topic>L-Lactate dehydrogenase</topic><topic>L-Lactate Dehydrogenase - metabolism</topic><topic>Lactate dehydrogenase</topic><topic>Lactic acid</topic><topic>Leukocytes (granulocytic)</topic><topic>Male</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Middle Aged</topic><topic>Oncology</topic><topic>Original Article</topic><topic>Pain</topic><topic>Paroxysmal nocturnal hemoglobinuria</topic><topic>Registries</topic><topic>Signs and symptoms</topic><topic>Thromboembolism</topic><topic>Thromboembolism - epidemiology</topic><topic>Thromboembolism - etiology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Sakurai, Masatoshi</creatorcontrib><creatorcontrib>Jang, Jun Ho</creatorcontrib><creatorcontrib>Chou, Wen-Chien</creatorcontrib><creatorcontrib>Kim, Jin Seok</creatorcontrib><creatorcontrib>Wilson, Amanda</creatorcontrib><creatorcontrib>Nishimura, Jun-ichi</creatorcontrib><creatorcontrib>Chiou, Tzeon-Jye</creatorcontrib><creatorcontrib>Kanakura, Yuzuru</creatorcontrib><creatorcontrib>Lee, Jong Wook</creatorcontrib><creatorcontrib>Okamoto, Shinichiro</creatorcontrib><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>Nursing & Allied Health Database</collection><collection>Immunology Abstracts</collection><collection>Industrial and Applied Microbiology Abstracts (Microbiology A)</collection><collection>Nucleic Acids Abstracts</collection><collection>ProQuest Health and Medical</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>ProQuest Pharma Collection</collection><collection>Technology Research Database</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)</collection><collection>ProQuest Central</collection><collection>ProQuest Central</collection><collection>Environmental Sciences and Pollution Management</collection><collection>ProQuest One Community College</collection><collection>Engineering Research Database</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Database (Alumni Edition)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical 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>MEDLINE - Academic</collection><jtitle>International journal of hematology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Sakurai, Masatoshi</au><au>Jang, Jun Ho</au><au>Chou, Wen-Chien</au><au>Kim, Jin Seok</au><au>Wilson, Amanda</au><au>Nishimura, Jun-ichi</au><au>Chiou, Tzeon-Jye</au><au>Kanakura, Yuzuru</au><au>Lee, Jong Wook</au><au>Okamoto, Shinichiro</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Comparative study on baseline clinical characteristics of Asian versus non-Asian patients with paroxysmal nocturnal hemoglobinuria</atitle><jtitle>International journal of hematology</jtitle><stitle>Int J Hematol</stitle><addtitle>Int J Hematol</addtitle><date>2019-10-01</date><risdate>2019</risdate><volume>110</volume><issue>4</issue><spage>411</spage><epage>418</epage><pages>411-418</pages><issn>0925-5710</issn><eissn>1865-3774</eissn><abstract>A difference in clinical manifestations of paroxysmal nocturnal hemoglobinuria (PNH) among different races has been suggested. The aim of this study was to clarify whether the clinical characteristics of patients with PNH in the International PNH Registry differ by ethnic background. Patients, who were eculizumab naïve at baseline and had ≥ 1% PNH clone size, were eligible for this analysis. Totally, 1793 patients were enrolled and divided into two cohorts, Asian (
N
= 246) and non-Asian (
N
= 1547). The Asian cohort was further divided into Asians in Asia cohort (
N
= 202) and Asians in non-Asia cohort (
N
= 44), based on geographical region. The Asian cohort had significantly higher PNH clone size in granulocytes, higher lactate dehydrogenase levels, and lower hemoglobin levels. However, the frequencies of symptoms including abdominal pain, backache, easy bleeding, fatigue and headache at baseline were significantly lower in the Asian cohort. The proportion of patients with a history of thromboembolism (TE) was significantly lower in the Asian than in the non-Asian cohort (3.6% vs. 8.9%,
P
< 0.01); however, there was no difference between Asians in Asia and Asians in non-Asia (3.3% vs. 4.9%,
P
= 0.61). These findings suggested that genetic factors may play a stronger role in developing TE than lifestyle factors in PNH patients.</abstract><cop>Tokyo</cop><pub>Springer Japan</pub><pmid>31278635</pmid><doi>10.1007/s12185-019-02699-7</doi><tpages>8</tpages></addata></record> |
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subjects | Abdominal Pain - epidemiology Abdominal Pain - etiology Adult Asian Continental Ancestry Group Asian people Back Pain - epidemiology Back Pain - etiology Bleeding Cloning Cohort Studies Comparative studies Continental Population Groups Fatigue - epidemiology Fatigue - etiology Female Genetic factors Granulocytes - pathology Headache Headache - epidemiology Headache - etiology Hematology Hemoglobin Hemoglobins Hemoglobinuria, Paroxysmal - blood Hemoglobinuria, Paroxysmal - complications Hemoglobinuria, Paroxysmal - genetics Hemoglobinuria, Paroxysmal - physiopathology Hemorrhage - epidemiology Hemorrhage - etiology Humans L-Lactate dehydrogenase L-Lactate Dehydrogenase - metabolism Lactate dehydrogenase Lactic acid Leukocytes (granulocytic) Male Medicine Medicine & Public Health Middle Aged Oncology Original Article Pain Paroxysmal nocturnal hemoglobinuria Registries Signs and symptoms Thromboembolism Thromboembolism - epidemiology Thromboembolism - etiology |
title | Comparative study on baseline clinical characteristics of Asian versus non-Asian patients with paroxysmal nocturnal hemoglobinuria |
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