Childhood chronic immune thrombocytopenic purpura: unresolved issues
Chronic immune thrombocytopenic purpura (ITP), defined as a platelet count of below 150 x 109/L persisting for more than 6 months from onset of illness, occurs in approximately 20% to 25% of children with acute-onset ITP. A small subset of these patients (approximately 5%) will manifest symptomatic,...
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Veröffentlicht in: | Journal of pediatric hematology/oncology 2003-12, Vol.25 Suppl 1 (Supplement 1), p.S28-S33 |
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container_title | Journal of pediatric hematology/oncology |
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creator | Blanchette, Victor S Price, Victoria |
description | Chronic immune thrombocytopenic purpura (ITP), defined as a platelet count of below 150 x 109/L persisting for more than 6 months from onset of illness, occurs in approximately 20% to 25% of children with acute-onset ITP. A small subset of these patients (approximately 5%) will manifest symptomatic, severe thrombocytopenia (platelet counts |
doi_str_mv | 10.1097/00043426-200312001-00007 |
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A small subset of these patients (approximately 5%) will manifest symptomatic, severe thrombocytopenia (platelet counts <20 x 109/L) at 1 year or longer following diagnosis, and may require splenectomy. Complete/partial response rates following splenectomy in children with primary chronic ITP are of the order of 70% to 75%; response rates are lower in children with secondary ITP and those with complex autoimmune cytopenias (e.g., Evans syndrome). Laparoscopic splenectomy is increasingly preferred over open splenectomy. Patients should be immunized with the pneumococcal, Haemophilus type b and meningococcal vaccines before splenectomy; the duration of postsplenectomy antibiotic prophylaxis using penicillin or an equivalent antibiotic is controversial but should be at least until 5 years of age and for a minimum of 1 year postsplenectomy. Some experts advocate life-long antibiotic prophylaxis. Treatment of postsplenectomy failures is a challenge; partial/complete remission rates are low, and multimodality therapy may be more efficacious than monotherapy. The presence of an accessory spleen should be sought and removal considered if present. The role of newer treatment modalities such as anti-CD 20 remains to be established.</description><identifier>ISSN: 1077-4114</identifier><identifier>DOI: 10.1097/00043426-200312001-00007</identifier><identifier>PMID: 14668636</identifier><language>eng</language><publisher>United States</publisher><subject>Age of Onset ; Child ; Child, Preschool ; Emergencies ; Female ; Humans ; Male ; Platelet Count ; Purpura, Thrombocytopenic, Idiopathic - blood ; Purpura, Thrombocytopenic, Idiopathic - physiopathology ; Purpura, Thrombocytopenic, Idiopathic - surgery ; Splenectomy</subject><ispartof>Journal of pediatric hematology/oncology, 2003-12, Vol.25 Suppl 1 (Supplement 1), p.S28-S33</ispartof><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c377t-2910fdc7d2974c7cd844823cebf5583f6aa619eaf8fa65a9e0a078dce84335d03</citedby><cites>FETCH-LOGICAL-c377t-2910fdc7d2974c7cd844823cebf5583f6aa619eaf8fa65a9e0a078dce84335d03</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>315,781,785,27929,27930</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/14668636$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Blanchette, Victor S</creatorcontrib><creatorcontrib>Price, Victoria</creatorcontrib><title>Childhood chronic immune thrombocytopenic purpura: unresolved issues</title><title>Journal of pediatric hematology/oncology</title><addtitle>J Pediatr Hematol Oncol</addtitle><description>Chronic immune thrombocytopenic purpura (ITP), defined as a platelet count of below 150 x 109/L persisting for more than 6 months from onset of illness, occurs in approximately 20% to 25% of children with acute-onset ITP. A small subset of these patients (approximately 5%) will manifest symptomatic, severe thrombocytopenia (platelet counts <20 x 109/L) at 1 year or longer following diagnosis, and may require splenectomy. Complete/partial response rates following splenectomy in children with primary chronic ITP are of the order of 70% to 75%; response rates are lower in children with secondary ITP and those with complex autoimmune cytopenias (e.g., Evans syndrome). Laparoscopic splenectomy is increasingly preferred over open splenectomy. Patients should be immunized with the pneumococcal, Haemophilus type b and meningococcal vaccines before splenectomy; the duration of postsplenectomy antibiotic prophylaxis using penicillin or an equivalent antibiotic is controversial but should be at least until 5 years of age and for a minimum of 1 year postsplenectomy. Some experts advocate life-long antibiotic prophylaxis. Treatment of postsplenectomy failures is a challenge; partial/complete remission rates are low, and multimodality therapy may be more efficacious than monotherapy. The presence of an accessory spleen should be sought and removal considered if present. The role of newer treatment modalities such as anti-CD 20 remains to be established.</description><subject>Age of Onset</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Emergencies</subject><subject>Female</subject><subject>Humans</subject><subject>Male</subject><subject>Platelet Count</subject><subject>Purpura, Thrombocytopenic, Idiopathic - blood</subject><subject>Purpura, Thrombocytopenic, Idiopathic - physiopathology</subject><subject>Purpura, Thrombocytopenic, Idiopathic - surgery</subject><subject>Splenectomy</subject><issn>1077-4114</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2003</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpFkMFOwzAMhnMAsTF4BdQTt4LTpEnKDQ0GSJO4wDnKElcrapuStEh7ezI2QLJs2f5_W_oIySjcUKjkLQBwxguRFwCMpkTzNAJ5QuYUpMw5pXxGzmP8SCuZlGdkRrkQSjAxJw_LbdO6rfcus9vg-8ZmTddNPWZjaruNt7vRD7ifD1NIYe6yqQ8YffuFLmtinDBekNPatBEvj3VB3lePb8vnfP369LK8X-eWSTnmRUWhdla6opLcSusU56pgFjd1WSpWC2MErdDUqjaiNBWCAamcRcUZKx2wBbk-3B2C_0x_R9010WLbmh79FLWkJTCQVRKqg9AGH2PAWg-h6UzYaQp6T03_UtN_1PQPtWS9Ov6YNh26f-MRGfsGdK9rEw</recordid><startdate>200312</startdate><enddate>200312</enddate><creator>Blanchette, Victor S</creator><creator>Price, Victoria</creator><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>7X8</scope></search><sort><creationdate>200312</creationdate><title>Childhood chronic immune thrombocytopenic purpura: unresolved issues</title><author>Blanchette, Victor S ; Price, Victoria</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c377t-2910fdc7d2974c7cd844823cebf5583f6aa619eaf8fa65a9e0a078dce84335d03</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2003</creationdate><topic>Age of Onset</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Emergencies</topic><topic>Female</topic><topic>Humans</topic><topic>Male</topic><topic>Platelet Count</topic><topic>Purpura, Thrombocytopenic, Idiopathic - blood</topic><topic>Purpura, Thrombocytopenic, Idiopathic - physiopathology</topic><topic>Purpura, Thrombocytopenic, Idiopathic - surgery</topic><topic>Splenectomy</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Blanchette, Victor S</creatorcontrib><creatorcontrib>Price, Victoria</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Journal of pediatric hematology/oncology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Blanchette, Victor S</au><au>Price, Victoria</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Childhood chronic immune thrombocytopenic purpura: unresolved issues</atitle><jtitle>Journal of pediatric hematology/oncology</jtitle><addtitle>J Pediatr Hematol Oncol</addtitle><date>2003-12</date><risdate>2003</risdate><volume>25 Suppl 1</volume><issue>Supplement 1</issue><spage>S28</spage><epage>S33</epage><pages>S28-S33</pages><issn>1077-4114</issn><abstract>Chronic immune thrombocytopenic purpura (ITP), defined as a platelet count of below 150 x 109/L persisting for more than 6 months from onset of illness, occurs in approximately 20% to 25% of children with acute-onset ITP. A small subset of these patients (approximately 5%) will manifest symptomatic, severe thrombocytopenia (platelet counts <20 x 109/L) at 1 year or longer following diagnosis, and may require splenectomy. Complete/partial response rates following splenectomy in children with primary chronic ITP are of the order of 70% to 75%; response rates are lower in children with secondary ITP and those with complex autoimmune cytopenias (e.g., Evans syndrome). Laparoscopic splenectomy is increasingly preferred over open splenectomy. Patients should be immunized with the pneumococcal, Haemophilus type b and meningococcal vaccines before splenectomy; the duration of postsplenectomy antibiotic prophylaxis using penicillin or an equivalent antibiotic is controversial but should be at least until 5 years of age and for a minimum of 1 year postsplenectomy. Some experts advocate life-long antibiotic prophylaxis. Treatment of postsplenectomy failures is a challenge; partial/complete remission rates are low, and multimodality therapy may be more efficacious than monotherapy. The presence of an accessory spleen should be sought and removal considered if present. The role of newer treatment modalities such as anti-CD 20 remains to be established.</abstract><cop>United States</cop><pmid>14668636</pmid><doi>10.1097/00043426-200312001-00007</doi></addata></record> |
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subjects | Age of Onset Child Child, Preschool Emergencies Female Humans Male Platelet Count Purpura, Thrombocytopenic, Idiopathic - blood Purpura, Thrombocytopenic, Idiopathic - physiopathology Purpura, Thrombocytopenic, Idiopathic - surgery Splenectomy |
title | Childhood chronic immune thrombocytopenic purpura: unresolved issues |
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