Acquired idiopathic ADAMTS13 activity deficient thrombotic thrombocytopenic purpura in a population from Japan
Thrombotic thrombocytopenic purpura (TTP) is a type of thrombotic microangiopathy (TMA). Studies report that the majority of TTP patients present with a deficiency of ADAMTS13 activity. In a database of TMA patients in Japan identified between 1998 and 2008, 186 patients with first onset of acquired...
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| Veröffentlicht in: | PloS one 2012-03, Vol.7 (3), p.e33029-e33029 |
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| creator | Matsumoto, Masanori Bennett, Charles L Isonishi, Ayami Qureshi, Zaina Hori, Yuji Hayakawa, Masaki Yoshida, Yoko Yagi, Hideo Fujimura, Yoshihiro |
| description | Thrombotic thrombocytopenic purpura (TTP) is a type of thrombotic microangiopathy (TMA). Studies report that the majority of TTP patients present with a deficiency of ADAMTS13 activity. In a database of TMA patients in Japan identified between 1998 and 2008, 186 patients with first onset of acquired idiopathic (ai) ADAMTS13-deficient TTP (ADAMTS13 activity |
| doi_str_mv | 10.1371/journal.pone.0033029 |
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Studies report that the majority of TTP patients present with a deficiency of ADAMTS13 activity. In a database of TMA patients in Japan identified between 1998 and 2008, 186 patients with first onset of acquired idiopathic (ai) ADAMTS13-deficient TTP (ADAMTS13 activity <5%) were diagnosed. The median age of onset of TTP in this group of patients was 54 years, 54.8% were female, 75.8% had renal involvement, 79.0% had neurologic symptoms, and 97.8% had detectable inhibitors to ADAMTS13 activity. Younger patients were less likely to present with renal or neurologic dysfunction (p<0.01), while older patients were more likely to die during the TTP hospitalization (p<0.05). Findings from this cohort in Japan differ from those reported previously from the United States, Europe, and Korea with respect to age at onset (two decades younger in the other cohort) and gender composition (60% to 100% female in the other cohort). We conclude that in one of the largest cohorts of ai-TTP with severe deficiency of ADAMTS13 activity reported to date, demographic characteristics differ in Japanese patients relative to those reported from a large Caucasian registry from Western societies. Additional studies exploring these findings are needed.</description><identifier>ISSN: 1932-6203</identifier><identifier>EISSN: 1932-6203</identifier><identifier>DOI: 10.1371/journal.pone.0033029</identifier><identifier>PMID: 22427934</identifier><language>eng</language><publisher>United States: Public Library of Science</publisher><subject>ADAM Proteins - blood ; ADAM Proteins - deficiency ; ADAMTS13 Protein ; Age ; Age Factors ; Biology ; Blood transfusions ; Cohort Studies ; Congenital diseases ; Demographics ; Enzymes ; Female ; Females ; Health risk assessment ; Humans ; Immunoassay ; Immunoglobulins ; Japan - epidemiology ; Kidneys ; Laboratories ; Male ; Medical research ; Medicine ; Middle Aged ; Mortality ; Patients ; Pharmacy ; Purpura ; Purpura, Thrombocytopenic, Idiopathic - epidemiology ; Purpura, Thrombocytopenic, Idiopathic - metabolism ; Purpura, Thrombocytopenic, Idiopathic - pathology ; Renal function ; Statistics, Nonparametric ; Studies ; Thrombocytopenic purpura ; Thrombotic microangiopathy ; Thrombotic thrombocytopenic purpura</subject><ispartof>PloS one, 2012-03, Vol.7 (3), p.e33029-e33029</ispartof><rights>COPYRIGHT 2012 Public Library of Science</rights><rights>2012 Matsumoto et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License: https://creativecommons.org/licenses/by/4.0/ (the “License”), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><rights>Matsumoto et al. 2012</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c757t-7b9dce6dc99d4217a0f984ca4331b5be38017db63e2226eb0f93113ae1c13a843</citedby><cites>FETCH-LOGICAL-c757t-7b9dce6dc99d4217a0f984ca4331b5be38017db63e2226eb0f93113ae1c13a843</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC3299727/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC3299727/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,723,776,780,860,881,2095,2914,23846,27903,27904,53770,53772,79347,79348</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/22427934$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><contributor>Reitsma, Pieter H.</contributor><creatorcontrib>Matsumoto, Masanori</creatorcontrib><creatorcontrib>Bennett, Charles L</creatorcontrib><creatorcontrib>Isonishi, Ayami</creatorcontrib><creatorcontrib>Qureshi, Zaina</creatorcontrib><creatorcontrib>Hori, Yuji</creatorcontrib><creatorcontrib>Hayakawa, Masaki</creatorcontrib><creatorcontrib>Yoshida, Yoko</creatorcontrib><creatorcontrib>Yagi, Hideo</creatorcontrib><creatorcontrib>Fujimura, Yoshihiro</creatorcontrib><title>Acquired idiopathic ADAMTS13 activity deficient thrombotic thrombocytopenic purpura in a population from Japan</title><title>PloS one</title><addtitle>PLoS One</addtitle><description>Thrombotic thrombocytopenic purpura (TTP) is a type of thrombotic microangiopathy (TMA). Studies report that the majority of TTP patients present with a deficiency of ADAMTS13 activity. In a database of TMA patients in Japan identified between 1998 and 2008, 186 patients with first onset of acquired idiopathic (ai) ADAMTS13-deficient TTP (ADAMTS13 activity <5%) were diagnosed. The median age of onset of TTP in this group of patients was 54 years, 54.8% were female, 75.8% had renal involvement, 79.0% had neurologic symptoms, and 97.8% had detectable inhibitors to ADAMTS13 activity. Younger patients were less likely to present with renal or neurologic dysfunction (p<0.01), while older patients were more likely to die during the TTP hospitalization (p<0.05). Findings from this cohort in Japan differ from those reported previously from the United States, Europe, and Korea with respect to age at onset (two decades younger in the other cohort) and gender composition (60% to 100% female in the other cohort). We conclude that in one of the largest cohorts of ai-TTP with severe deficiency of ADAMTS13 activity reported to date, demographic characteristics differ in Japanese patients relative to those reported from a large Caucasian registry from Western societies. Additional studies exploring these findings are needed.</description><subject>ADAM Proteins - blood</subject><subject>ADAM Proteins - deficiency</subject><subject>ADAMTS13 Protein</subject><subject>Age</subject><subject>Age Factors</subject><subject>Biology</subject><subject>Blood transfusions</subject><subject>Cohort Studies</subject><subject>Congenital diseases</subject><subject>Demographics</subject><subject>Enzymes</subject><subject>Female</subject><subject>Females</subject><subject>Health risk assessment</subject><subject>Humans</subject><subject>Immunoassay</subject><subject>Immunoglobulins</subject><subject>Japan - epidemiology</subject><subject>Kidneys</subject><subject>Laboratories</subject><subject>Male</subject><subject>Medical research</subject><subject>Medicine</subject><subject>Middle Aged</subject><subject>Mortality</subject><subject>Patients</subject><subject>Pharmacy</subject><subject>Purpura</subject><subject>Purpura, Thrombocytopenic, Idiopathic - epidemiology</subject><subject>Purpura, Thrombocytopenic, Idiopathic - metabolism</subject><subject>Purpura, Thrombocytopenic, Idiopathic - pathology</subject><subject>Renal function</subject><subject>Statistics, Nonparametric</subject><subject>Studies</subject><subject>Thrombocytopenic purpura</subject><subject>Thrombotic microangiopathy</subject><subject>Thrombotic thrombocytopenic purpura</subject><issn>1932-6203</issn><issn>1932-6203</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><sourceid>DOA</sourceid><recordid>eNqNk9-P1CAQxxuj8c7V_8BoExOND7sC05bycsnm_LXmzCXe6SuhlO6y6UIP6MX976Vu77I192AgQIbPfGEGJkleYrTAQPGHre2dEe2is0YtEAJAhD1KTjEDMi8IgsdH65PkmfdbhHIoi-JpckJIRiiD7DQxS3nTa6fqVNfadiJstEyXH5ffr68wpEIGfavDPq1Vo6VWJqRh4-yusiFi41Lug-2UiYaud7GLVJtUpJ3t-lYEbU3aRC79JjphnidPGtF69WKcZ8nPz5-uz7_OLy6_rM6XF3NJcxrmtGK1VEUtGaszgqlADSszKTIAXOWVghJhWlcFKEJIoaq4DRiDUFjGscxglrw-6Hat9XxMlecYCDAGLOrMktWBqK3Y8s7pnXB7boXmfw3WrblwMcpWcdyUlGJADCqZ5aqsclmSvJJ5U1elLFjUOhtP66udijc3wYl2IjrdMXrD1_aWA2GMEhoF3o0Czt70yge-016qthVG2d5zRhgmhNIhsDf_kA8HN1JrEe-vTWPjsXLQ5MtsiAXKEkdq8QAVW612WsZv1ehonzi8nzhEJqjfYS167_nq6sf_s5e_puzbI3ajRBs23rb98Hn8FMwOoHTWe6ea-xxjxIequMsGH6qCj1UR3V4dv8-9010ZwB8P7Qgx</recordid><startdate>20120312</startdate><enddate>20120312</enddate><creator>Matsumoto, Masanori</creator><creator>Bennett, Charles L</creator><creator>Isonishi, Ayami</creator><creator>Qureshi, Zaina</creator><creator>Hori, Yuji</creator><creator>Hayakawa, Masaki</creator><creator>Yoshida, Yoko</creator><creator>Yagi, Hideo</creator><creator>Fujimura, Yoshihiro</creator><general>Public Library of Science</general><general>Public Library of Science (PLoS)</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>IOV</scope><scope>ISR</scope><scope>3V.</scope><scope>7QG</scope><scope>7QL</scope><scope>7QO</scope><scope>7RV</scope><scope>7SN</scope><scope>7SS</scope><scope>7T5</scope><scope>7TG</scope><scope>7TM</scope><scope>7U9</scope><scope>7X2</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8C1</scope><scope>8FD</scope><scope>8FE</scope><scope>8FG</scope><scope>8FH</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABJCF</scope><scope>ABUWG</scope><scope>AEUYN</scope><scope>AFKRA</scope><scope>ARAPS</scope><scope>ATCPS</scope><scope>AZQEC</scope><scope>BBNVY</scope><scope>BENPR</scope><scope>BGLVJ</scope><scope>BHPHI</scope><scope>C1K</scope><scope>CCPQU</scope><scope>D1I</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>KB.</scope><scope>KB0</scope><scope>KL.</scope><scope>L6V</scope><scope>LK8</scope><scope>M0K</scope><scope>M0S</scope><scope>M1P</scope><scope>M7N</scope><scope>M7P</scope><scope>M7S</scope><scope>NAPCQ</scope><scope>P5Z</scope><scope>P62</scope><scope>P64</scope><scope>PATMY</scope><scope>PDBOC</scope><scope>PIMPY</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>PTHSS</scope><scope>PYCSY</scope><scope>RC3</scope><scope>7X8</scope><scope>5PM</scope><scope>DOA</scope></search><sort><creationdate>20120312</creationdate><title>Acquired idiopathic ADAMTS13 activity deficient thrombotic thrombocytopenic purpura in a population from Japan</title><author>Matsumoto, Masanori ; Bennett, Charles L ; Isonishi, Ayami ; Qureshi, Zaina ; Hori, Yuji ; Hayakawa, Masaki ; Yoshida, Yoko ; Yagi, Hideo ; Fujimura, Yoshihiro</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c757t-7b9dce6dc99d4217a0f984ca4331b5be38017db63e2226eb0f93113ae1c13a843</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2012</creationdate><topic>ADAM Proteins - blood</topic><topic>ADAM Proteins - deficiency</topic><topic>ADAMTS13 Protein</topic><topic>Age</topic><topic>Age Factors</topic><topic>Biology</topic><topic>Blood transfusions</topic><topic>Cohort Studies</topic><topic>Congenital diseases</topic><topic>Demographics</topic><topic>Enzymes</topic><topic>Female</topic><topic>Females</topic><topic>Health risk assessment</topic><topic>Humans</topic><topic>Immunoassay</topic><topic>Immunoglobulins</topic><topic>Japan - epidemiology</topic><topic>Kidneys</topic><topic>Laboratories</topic><topic>Male</topic><topic>Medical research</topic><topic>Medicine</topic><topic>Middle Aged</topic><topic>Mortality</topic><topic>Patients</topic><topic>Pharmacy</topic><topic>Purpura</topic><topic>Purpura, Thrombocytopenic, Idiopathic - epidemiology</topic><topic>Purpura, Thrombocytopenic, Idiopathic - metabolism</topic><topic>Purpura, Thrombocytopenic, Idiopathic - pathology</topic><topic>Renal function</topic><topic>Statistics, Nonparametric</topic><topic>Studies</topic><topic>Thrombocytopenic purpura</topic><topic>Thrombotic microangiopathy</topic><topic>Thrombotic thrombocytopenic purpura</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Matsumoto, Masanori</creatorcontrib><creatorcontrib>Bennett, Charles L</creatorcontrib><creatorcontrib>Isonishi, Ayami</creatorcontrib><creatorcontrib>Qureshi, Zaina</creatorcontrib><creatorcontrib>Hori, Yuji</creatorcontrib><creatorcontrib>Hayakawa, Masaki</creatorcontrib><creatorcontrib>Yoshida, Yoko</creatorcontrib><creatorcontrib>Yagi, Hideo</creatorcontrib><creatorcontrib>Fujimura, Yoshihiro</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Gale In Context: Opposing Viewpoints</collection><collection>Gale In Context: Science</collection><collection>ProQuest Central (Corporate)</collection><collection>Animal Behavior Abstracts</collection><collection>Bacteriology Abstracts (Microbiology B)</collection><collection>Biotechnology Research Abstracts</collection><collection>Nursing & Allied Health Database</collection><collection>Ecology Abstracts</collection><collection>Entomology Abstracts (Full archive)</collection><collection>Immunology Abstracts</collection><collection>Meteorological & Geoastrophysical Abstracts</collection><collection>Nucleic Acids Abstracts</collection><collection>Virology and AIDS Abstracts</collection><collection>Agricultural Science Collection</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 Technology 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>Materials Science & Engineering Collection</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest One Sustainability</collection><collection>ProQuest Central UK/Ireland</collection><collection>Advanced Technologies & Aerospace Collection</collection><collection>Agricultural & Environmental Science Collection</collection><collection>ProQuest Central Essentials</collection><collection>Biological Science Collection</collection><collection>ProQuest Central</collection><collection>Technology Collection</collection><collection>Natural Science Collection</collection><collection>Environmental Sciences and Pollution Management</collection><collection>ProQuest One Community College</collection><collection>ProQuest Materials Science Collection</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>Materials Science Database</collection><collection>Nursing & Allied Health Database (Alumni Edition)</collection><collection>Meteorological & Geoastrophysical Abstracts - 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Academic</collection><collection>PubMed Central (Full Participant titles)</collection><collection>DOAJ Directory of Open Access Journals</collection><jtitle>PloS one</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Matsumoto, Masanori</au><au>Bennett, Charles L</au><au>Isonishi, Ayami</au><au>Qureshi, Zaina</au><au>Hori, Yuji</au><au>Hayakawa, Masaki</au><au>Yoshida, Yoko</au><au>Yagi, Hideo</au><au>Fujimura, Yoshihiro</au><au>Reitsma, Pieter H.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Acquired idiopathic ADAMTS13 activity deficient thrombotic thrombocytopenic purpura in a population from Japan</atitle><jtitle>PloS one</jtitle><addtitle>PLoS One</addtitle><date>2012-03-12</date><risdate>2012</risdate><volume>7</volume><issue>3</issue><spage>e33029</spage><epage>e33029</epage><pages>e33029-e33029</pages><issn>1932-6203</issn><eissn>1932-6203</eissn><abstract>Thrombotic thrombocytopenic purpura (TTP) is a type of thrombotic microangiopathy (TMA). Studies report that the majority of TTP patients present with a deficiency of ADAMTS13 activity. In a database of TMA patients in Japan identified between 1998 and 2008, 186 patients with first onset of acquired idiopathic (ai) ADAMTS13-deficient TTP (ADAMTS13 activity <5%) were diagnosed. The median age of onset of TTP in this group of patients was 54 years, 54.8% were female, 75.8% had renal involvement, 79.0% had neurologic symptoms, and 97.8% had detectable inhibitors to ADAMTS13 activity. Younger patients were less likely to present with renal or neurologic dysfunction (p<0.01), while older patients were more likely to die during the TTP hospitalization (p<0.05). Findings from this cohort in Japan differ from those reported previously from the United States, Europe, and Korea with respect to age at onset (two decades younger in the other cohort) and gender composition (60% to 100% female in the other cohort). We conclude that in one of the largest cohorts of ai-TTP with severe deficiency of ADAMTS13 activity reported to date, demographic characteristics differ in Japanese patients relative to those reported from a large Caucasian registry from Western societies. Additional studies exploring these findings are needed.</abstract><cop>United States</cop><pub>Public Library of Science</pub><pmid>22427934</pmid><doi>10.1371/journal.pone.0033029</doi><tpages>e33029</tpages><oa>free_for_read</oa></addata></record> |
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| subjects | ADAM Proteins - blood ADAM Proteins - deficiency ADAMTS13 Protein Age Age Factors Biology Blood transfusions Cohort Studies Congenital diseases Demographics Enzymes Female Females Health risk assessment Humans Immunoassay Immunoglobulins Japan - epidemiology Kidneys Laboratories Male Medical research Medicine Middle Aged Mortality Patients Pharmacy Purpura Purpura, Thrombocytopenic, Idiopathic - epidemiology Purpura, Thrombocytopenic, Idiopathic - metabolism Purpura, Thrombocytopenic, Idiopathic - pathology Renal function Statistics, Nonparametric Studies Thrombocytopenic purpura Thrombotic microangiopathy Thrombotic thrombocytopenic purpura |
| title | Acquired idiopathic ADAMTS13 activity deficient thrombotic thrombocytopenic purpura in a population from Japan |
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