Thrombotic Microangiopathic Hemolytic Anemia With Reduction of ADAMTS13 Activity: Initial Manifestation of Childhood-Onset Systemic Lupus Erythematosus
Severe manifestations of systemic lupus erythematosus (SLE), antiphospholipid syndrome (APS), and thrombotic thrombocytopenic purpura (TTP) are characterized by multiorgan thrombotic microangiopathy. We describe reduction of ADAMTS13 activity and the development of systemic autoimmunity in all 8 chi...
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| Veröffentlicht in: | American journal of clinical pathology 2011-03, Vol.135 (3), p.406-416 |
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| creator | MUSCAL, Eyal EDWARDS, Rachel M KEARNEY, Debra L HICKS, John M MYONES, Barry L TERUYA, Jun |
| description | Severe manifestations of systemic lupus erythematosus (SLE), antiphospholipid syndrome (APS), and thrombotic thrombocytopenic purpura (TTP) are characterized by multiorgan thrombotic microangiopathy. We describe reduction of ADAMTS13 activity and the development of systemic autoimmunity in all 8 children initially diagnosed with acquired noncongenital TTP during an 8.5-year period. Median age at diagnosis was 12.0 years (range, 2.6-17.3 years). ADAMTS13 activity was absent ( |
| doi_str_mv | 10.1309/AJCP5BVL4FCLCGLU |
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We describe reduction of ADAMTS13 activity and the development of systemic autoimmunity in all 8 children initially diagnosed with acquired noncongenital TTP during an 8.5-year period. Median age at diagnosis was 12.0 years (range, 2.6-17.3 years). ADAMTS13 activity was absent (<5%) in 6 patients; 3 patients had a detected inhibitor. SLE was diagnosed concurrently in 3 patients, and 4 patients were diagnosed within 5 years. Six of the children diagnosed with SLE had absent ADAMTS13 activity at diagnosis. In 6 patients with SLE, immune-mediated nephritis developed by 46 months. All surviving patients with SLE developed antiphospholipid antibodies, including some with a lupus anticoagulant. Patients with SLE did not have TTP recurrences once daily immunosuppressive regimens were started. An evaluation for SLE/APS is warranted in children and adolescents with reduced ADAMTS13 activity and thrombotic microangiopathy.</description><identifier>ISSN: 0002-9173</identifier><identifier>EISSN: 1943-7722</identifier><identifier>DOI: 10.1309/AJCP5BVL4FCLCGLU</identifier><identifier>PMID: 21350095</identifier><identifier>CODEN: AJCPAI</identifier><language>eng</language><publisher>Chicago, IL: American Society of Clinical Pathologists</publisher><subject>ADAM Proteins - metabolism ; ADAMTS13 Protein ; Adolescent ; Anemias. Hemoglobinopathies ; Antibodies, Antiphospholipid - immunology ; Autoimmunity - immunology ; Biological and medical sciences ; Child ; Child, Preschool ; Diseases of red blood cells ; Female ; Glomerular Basement Membrane - immunology ; Glomerular Basement Membrane - ultrastructure ; Glomerulonephritis - complications ; Glomerulonephritis - immunology ; Glomerulonephritis - pathology ; Hematologic and hematopoietic diseases ; Humans ; Investigative techniques, diagnostic techniques (general aspects) ; Lupus Erythematosus, Systemic - complications ; Lupus Erythematosus, Systemic - metabolism ; Lupus Erythematosus, Systemic - pathology ; Male ; Medical sciences ; Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques ; Purpura, Thrombotic Thrombocytopenic - complications ; Purpura, Thrombotic Thrombocytopenic - metabolism ; Purpura, Thrombotic Thrombocytopenic - pathology</subject><ispartof>American journal of clinical pathology, 2011-03, Vol.135 (3), p.406-416</ispartof><rights>2015 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,27903,27904</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=23889830$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/21350095$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>MUSCAL, Eyal</creatorcontrib><creatorcontrib>EDWARDS, Rachel M</creatorcontrib><creatorcontrib>KEARNEY, Debra L</creatorcontrib><creatorcontrib>HICKS, John M</creatorcontrib><creatorcontrib>MYONES, Barry L</creatorcontrib><creatorcontrib>TERUYA, Jun</creatorcontrib><title>Thrombotic Microangiopathic Hemolytic Anemia With Reduction of ADAMTS13 Activity: Initial Manifestation of Childhood-Onset Systemic Lupus Erythematosus</title><title>American journal of clinical pathology</title><addtitle>Am J Clin Pathol</addtitle><description>Severe manifestations of systemic lupus erythematosus (SLE), antiphospholipid syndrome (APS), and thrombotic thrombocytopenic purpura (TTP) are characterized by multiorgan thrombotic microangiopathy. We describe reduction of ADAMTS13 activity and the development of systemic autoimmunity in all 8 children initially diagnosed with acquired noncongenital TTP during an 8.5-year period. Median age at diagnosis was 12.0 years (range, 2.6-17.3 years). ADAMTS13 activity was absent (<5%) in 6 patients; 3 patients had a detected inhibitor. SLE was diagnosed concurrently in 3 patients, and 4 patients were diagnosed within 5 years. Six of the children diagnosed with SLE had absent ADAMTS13 activity at diagnosis. In 6 patients with SLE, immune-mediated nephritis developed by 46 months. All surviving patients with SLE developed antiphospholipid antibodies, including some with a lupus anticoagulant. Patients with SLE did not have TTP recurrences once daily immunosuppressive regimens were started. An evaluation for SLE/APS is warranted in children and adolescents with reduced ADAMTS13 activity and thrombotic microangiopathy.</description><subject>ADAM Proteins - metabolism</subject><subject>ADAMTS13 Protein</subject><subject>Adolescent</subject><subject>Anemias. Hemoglobinopathies</subject><subject>Antibodies, Antiphospholipid - immunology</subject><subject>Autoimmunity - immunology</subject><subject>Biological and medical sciences</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Diseases of red blood cells</subject><subject>Female</subject><subject>Glomerular Basement Membrane - immunology</subject><subject>Glomerular Basement Membrane - ultrastructure</subject><subject>Glomerulonephritis - complications</subject><subject>Glomerulonephritis - immunology</subject><subject>Glomerulonephritis - pathology</subject><subject>Hematologic and hematopoietic diseases</subject><subject>Humans</subject><subject>Investigative techniques, diagnostic techniques (general aspects)</subject><subject>Lupus Erythematosus, Systemic - complications</subject><subject>Lupus Erythematosus, Systemic - metabolism</subject><subject>Lupus Erythematosus, Systemic - pathology</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques</subject><subject>Purpura, Thrombotic Thrombocytopenic - complications</subject><subject>Purpura, Thrombotic Thrombocytopenic - metabolism</subject><subject>Purpura, Thrombotic Thrombocytopenic - pathology</subject><issn>0002-9173</issn><issn>1943-7722</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2011</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpF0Etv1DAQB3CrAtGl5d4T8gVxCvi5ibmFsH2grIrotj2uZv0gRkkcYgcpn6RflyC24jIj_een0WgQuqDkA-VEfSy_Vt_k54daXFZ1dVXfn6AVVYJnec7YC7QihLBM0Zyfotcx_iSEsoKIV-iUUS4JUXKFnnbNGLpDSF7jrddjgP6HDwOkZgmubRfa-e-o7G3nAT_61ODv1kw6-dDj4HD5pdzu7ijH5RL99mn-hG96nzy0eAu9dzYmeLZV41vThGCy2z7ahO_mmJa1GtfTMEW8GefU2A5SiFM8Ry8dtNG-OfYzdH-52VXXWX17dVOVdTYwQVKWS6qtlrAWxVoAVQBrLYkT0ijI2YFwt1S7dlY5c6DaGaMKowgVzEJhmOBn6P2_vcMYfk3LtfvOR23bFnobprgvJFdKSKIW-fYop0NnzX4YfQfjvH_-5QLeHQFEDa0bodc-_ne8KFTBCf8DjCKHMw</recordid><startdate>20110301</startdate><enddate>20110301</enddate><creator>MUSCAL, Eyal</creator><creator>EDWARDS, Rachel M</creator><creator>KEARNEY, Debra L</creator><creator>HICKS, John M</creator><creator>MYONES, Barry L</creator><creator>TERUYA, Jun</creator><general>American Society of Clinical Pathologists</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>7X8</scope></search><sort><creationdate>20110301</creationdate><title>Thrombotic Microangiopathic Hemolytic Anemia With Reduction of ADAMTS13 Activity: Initial Manifestation of Childhood-Onset Systemic Lupus Erythematosus</title><author>MUSCAL, Eyal ; EDWARDS, Rachel M ; KEARNEY, Debra L ; HICKS, John M ; MYONES, Barry L ; TERUYA, Jun</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-p240t-751cec5a64864a19aa6c50f45d9a72b03f72be6fe9fdb1cfdd98d90142ea8d243</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2011</creationdate><topic>ADAM Proteins - metabolism</topic><topic>ADAMTS13 Protein</topic><topic>Adolescent</topic><topic>Anemias. Hemoglobinopathies</topic><topic>Antibodies, Antiphospholipid - immunology</topic><topic>Autoimmunity - immunology</topic><topic>Biological and medical sciences</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Diseases of red blood cells</topic><topic>Female</topic><topic>Glomerular Basement Membrane - immunology</topic><topic>Glomerular Basement Membrane - ultrastructure</topic><topic>Glomerulonephritis - complications</topic><topic>Glomerulonephritis - immunology</topic><topic>Glomerulonephritis - pathology</topic><topic>Hematologic and hematopoietic diseases</topic><topic>Humans</topic><topic>Investigative techniques, diagnostic techniques (general aspects)</topic><topic>Lupus Erythematosus, Systemic - complications</topic><topic>Lupus Erythematosus, Systemic - metabolism</topic><topic>Lupus Erythematosus, Systemic - pathology</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques</topic><topic>Purpura, Thrombotic Thrombocytopenic - complications</topic><topic>Purpura, Thrombotic Thrombocytopenic - metabolism</topic><topic>Purpura, Thrombotic Thrombocytopenic - pathology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>MUSCAL, Eyal</creatorcontrib><creatorcontrib>EDWARDS, Rachel M</creatorcontrib><creatorcontrib>KEARNEY, Debra L</creatorcontrib><creatorcontrib>HICKS, John M</creatorcontrib><creatorcontrib>MYONES, Barry L</creatorcontrib><creatorcontrib>TERUYA, Jun</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>MEDLINE - Academic</collection><jtitle>American journal of clinical pathology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>MUSCAL, Eyal</au><au>EDWARDS, Rachel M</au><au>KEARNEY, Debra L</au><au>HICKS, John M</au><au>MYONES, Barry L</au><au>TERUYA, Jun</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Thrombotic Microangiopathic Hemolytic Anemia With Reduction of ADAMTS13 Activity: Initial Manifestation of Childhood-Onset Systemic Lupus Erythematosus</atitle><jtitle>American journal of clinical pathology</jtitle><addtitle>Am J Clin Pathol</addtitle><date>2011-03-01</date><risdate>2011</risdate><volume>135</volume><issue>3</issue><spage>406</spage><epage>416</epage><pages>406-416</pages><issn>0002-9173</issn><eissn>1943-7722</eissn><coden>AJCPAI</coden><abstract>Severe manifestations of systemic lupus erythematosus (SLE), antiphospholipid syndrome (APS), and thrombotic thrombocytopenic purpura (TTP) are characterized by multiorgan thrombotic microangiopathy. We describe reduction of ADAMTS13 activity and the development of systemic autoimmunity in all 8 children initially diagnosed with acquired noncongenital TTP during an 8.5-year period. Median age at diagnosis was 12.0 years (range, 2.6-17.3 years). ADAMTS13 activity was absent (<5%) in 6 patients; 3 patients had a detected inhibitor. SLE was diagnosed concurrently in 3 patients, and 4 patients were diagnosed within 5 years. Six of the children diagnosed with SLE had absent ADAMTS13 activity at diagnosis. In 6 patients with SLE, immune-mediated nephritis developed by 46 months. All surviving patients with SLE developed antiphospholipid antibodies, including some with a lupus anticoagulant. Patients with SLE did not have TTP recurrences once daily immunosuppressive regimens were started. 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| ispartof | American journal of clinical pathology, 2011-03, Vol.135 (3), p.406-416 |
| issn | 0002-9173 1943-7722 |
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| source | MEDLINE; Oxford University Press Journals All Titles (1996-Current); EZB-FREE-00999 freely available EZB journals |
| subjects | ADAM Proteins - metabolism ADAMTS13 Protein Adolescent Anemias. Hemoglobinopathies Antibodies, Antiphospholipid - immunology Autoimmunity - immunology Biological and medical sciences Child Child, Preschool Diseases of red blood cells Female Glomerular Basement Membrane - immunology Glomerular Basement Membrane - ultrastructure Glomerulonephritis - complications Glomerulonephritis - immunology Glomerulonephritis - pathology Hematologic and hematopoietic diseases Humans Investigative techniques, diagnostic techniques (general aspects) Lupus Erythematosus, Systemic - complications Lupus Erythematosus, Systemic - metabolism Lupus Erythematosus, Systemic - pathology Male Medical sciences Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques Purpura, Thrombotic Thrombocytopenic - complications Purpura, Thrombotic Thrombocytopenic - metabolism Purpura, Thrombotic Thrombocytopenic - pathology |
| title | Thrombotic Microangiopathic Hemolytic Anemia With Reduction of ADAMTS13 Activity: Initial Manifestation of Childhood-Onset Systemic Lupus Erythematosus |
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