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Bleeding spectrum in children with moderate or severe von Willebrand disease: Relevance of pediatric‐specific bleeding

The bleeding phenotype of children with von Willebrand disease (VWD) needs to be characterized in detail to facilitate diagnosis during childhood and aid in the planning and assessment of treatment strategies. The objective was to evaluate the occurrence, type, and severity of bleeding in a large co...

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Published in:	American journal of hematology 2015-12, Vol.90 (12), p.1142-1148
Main Authors:	Sanders, Yvonne V., Fijnvandraat, Karin, Boender, Johan, Mauser‐Bunschoten, Evelien P., van der Bom, Johanna G., de Meris, Joke, Smiers, Frans J., Granzen, Bernd, Brons, Paul, Tamminga, Rienk Y.J., Cnossen, Marjon H., Leebeek, Frank W.G.
Format:	Article
Language:	English
Subjects:	Adolescent Adult Child Child, Preschool Cross-Sectional Studies Female Hemorrhage - genetics Humans Male von Willebrand Diseases - complications von Willebrand Diseases - diagnosis Young Adult
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creator	Sanders, Yvonne V. Fijnvandraat, Karin Boender, Johan Mauser‐Bunschoten, Evelien P. van der Bom, Johanna G. de Meris, Joke Smiers, Frans J. Granzen, Bernd Brons, Paul Tamminga, Rienk Y.J. Cnossen, Marjon H. Leebeek, Frank W.G.
description	The bleeding phenotype of children with von Willebrand disease (VWD) needs to be characterized in detail to facilitate diagnosis during childhood and aid in the planning and assessment of treatment strategies. The objective was to evaluate the occurrence, type, and severity of bleeding in a large cohort of children with moderate and severe VWD. We included 113 children (aged 0–16 years) with Type 1 (n = 60), 2 (n = 44), and 3 (n = 9) VWD with von Willebrand factor (VWF) antigen and/or VWF ristocetin cofactor levels ≤ 30 U/dL from a nation‐wide cross‐sectional study (“Willebrand in the Netherlands” study). Bleeding severity and frequency were determined using the International Society on Thrombosis and Hemostasis‐Bleeding Assessment Tool (ISTH‐BAT) with supplementary pediatric‐specific bleeding symptoms (umbilical stump bleeding, cephalohematoma, cheek hematoma, conjunctival bleeding, postcircumcision and postvenipuncture bleeding). We found that all 26 postmenarche girls experienced menorrhagia. Other common bleedings were cutaneous (81%), oropharyngeal (64%), prolonged bleeding from minor wounds (58%), and epistaxis (56%). Pediatric‐specific bleeding symptoms were present in 44% of patients. ISTH‐BAT bleeding score was higher in index cases than in affected family members (median, 12.0 vs. 6.5, P
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The objective was to evaluate the occurrence, type, and severity of bleeding in a large cohort of children with moderate and severe VWD. We included 113 children (aged 0–16 years) with Type 1 (n = 60), 2 (n = 44), and 3 (n = 9) VWD with von Willebrand factor (VWF) antigen and/or VWF ristocetin cofactor levels ≤ 30 U/dL from a nation‐wide cross‐sectional study (“Willebrand in the Netherlands” study). Bleeding severity and frequency were determined using the International Society on Thrombosis and Hemostasis‐Bleeding Assessment Tool (ISTH‐BAT) with supplementary pediatric‐specific bleeding symptoms (umbilical stump bleeding, cephalohematoma, cheek hematoma, conjunctival bleeding, postcircumcision and postvenipuncture bleeding). We found that all 26 postmenarche girls experienced menorrhagia. Other common bleedings were cutaneous (81%), oropharyngeal (64%), prolonged bleeding from minor wounds (58%), and epistaxis (56%). Pediatric‐specific bleeding symptoms were present in 44% of patients. ISTH‐BAT bleeding score was higher in index cases than in affected family members (median, 12.0 vs. 6.5, P < 0.001), higher in Type 3 VWD than in Type 2 or 1 (17.0 vs. 10.5 or 6.5, P < 0.001) and higher in children with severe (<10 U/dL) than moderate VWD (10–30 U/dL) (11.0 vs. 7.0, P < 0.001). Frequency of any bleeding, epistaxis, and oral cavity was higher in types 2 and 3 than in Type 1 VWD and was associated with VWF levels. We conclude that pediatric‐specific bleeding symptoms occurred in a large proportion of children with moderate or severe VWD and should be included when evaluating children for VWD. Am. J. Hematol. 90:1142–1148, 2015. © 2015 Wiley Periodicals, Inc.</description><identifier>ISSN: 0361-8609</identifier><identifier>EISSN: 1096-8652</identifier><identifier>DOI: 10.1002/ajh.24195</identifier><identifier>PMID: 26375306</identifier><language>eng</language><publisher>United States</publisher><subject>Adolescent ; Adult ; Child ; Child, Preschool ; Cross-Sectional Studies ; Female ; Hemorrhage - genetics ; Humans ; Male ; von Willebrand Diseases - complications ; von Willebrand Diseases - diagnosis ; Young Adult</subject><ispartof>American journal of hematology, 2015-12, Vol.90 (12), p.1142-1148</ispartof><rights>2015 Wiley Periodicals, Inc.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>315,783,787,27936,27937</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/26375306$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Sanders, Yvonne V.</creatorcontrib><creatorcontrib>Fijnvandraat, Karin</creatorcontrib><creatorcontrib>Boender, Johan</creatorcontrib><creatorcontrib>Mauser‐Bunschoten, Evelien P.</creatorcontrib><creatorcontrib>van der Bom, Johanna G.</creatorcontrib><creatorcontrib>de Meris, Joke</creatorcontrib><creatorcontrib>Smiers, Frans J.</creatorcontrib><creatorcontrib>Granzen, Bernd</creatorcontrib><creatorcontrib>Brons, Paul</creatorcontrib><creatorcontrib>Tamminga, Rienk Y.J.</creatorcontrib><creatorcontrib>Cnossen, Marjon H.</creatorcontrib><creatorcontrib>Leebeek, Frank W.G.</creatorcontrib><creatorcontrib>WiN Study Group</creatorcontrib><title>Bleeding spectrum in children with moderate or severe von Willebrand disease: Relevance of pediatric‐specific bleeding</title><title>American journal of hematology</title><addtitle>Am J Hematol</addtitle><description>The bleeding phenotype of children with von Willebrand disease (VWD) needs to be characterized in detail to facilitate diagnosis during childhood and aid in the planning and assessment of treatment strategies. The objective was to evaluate the occurrence, type, and severity of bleeding in a large cohort of children with moderate and severe VWD. We included 113 children (aged 0–16 years) with Type 1 (n = 60), 2 (n = 44), and 3 (n = 9) VWD with von Willebrand factor (VWF) antigen and/or VWF ristocetin cofactor levels ≤ 30 U/dL from a nation‐wide cross‐sectional study (“Willebrand in the Netherlands” study). Bleeding severity and frequency were determined using the International Society on Thrombosis and Hemostasis‐Bleeding Assessment Tool (ISTH‐BAT) with supplementary pediatric‐specific bleeding symptoms (umbilical stump bleeding, cephalohematoma, cheek hematoma, conjunctival bleeding, postcircumcision and postvenipuncture bleeding). We found that all 26 postmenarche girls experienced menorrhagia. Other common bleedings were cutaneous (81%), oropharyngeal (64%), prolonged bleeding from minor wounds (58%), and epistaxis (56%). Pediatric‐specific bleeding symptoms were present in 44% of patients. ISTH‐BAT bleeding score was higher in index cases than in affected family members (median, 12.0 vs. 6.5, P < 0.001), higher in Type 3 VWD than in Type 2 or 1 (17.0 vs. 10.5 or 6.5, P < 0.001) and higher in children with severe (<10 U/dL) than moderate VWD (10–30 U/dL) (11.0 vs. 7.0, P < 0.001). Frequency of any bleeding, epistaxis, and oral cavity was higher in types 2 and 3 than in Type 1 VWD and was associated with VWF levels. We conclude that pediatric‐specific bleeding symptoms occurred in a large proportion of children with moderate or severe VWD and should be included when evaluating children for VWD. Am. J. Hematol. 90:1142–1148, 2015. © 2015 Wiley Periodicals, Inc.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Cross-Sectional Studies</subject><subject>Female</subject><subject>Hemorrhage - genetics</subject><subject>Humans</subject><subject>Male</subject><subject>von Willebrand Diseases - complications</subject><subject>von Willebrand Diseases - diagnosis</subject><subject>Young Adult</subject><issn>0361-8609</issn><issn>1096-8652</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2015</creationdate><recordtype>article</recordtype><recordid>eNo9kctOwzAQRS0EglJY8APISzYBO46dhB1UPFUJCYFYWn5MqCvngZ0W2PEJfCNfQqCF1Vxpju5IcxA6oOSYEpKeqPnsOM1oyTfQiJJSJIXg6SYaESbokEm5g3ZjnBNCaVaQbbSTCpZzRsQIvZ17AOuaZxw7MH1Y1Ng12MyctwEa_Or6Ga5bC0H1gNuAIywhAF62DX5y3oMOqrHYuggqwim-Bw9L1ZiBrXA3FKs-OPP18fnT7ipnsF7f20NblfIR9tdzjB4vLx4m18n07upmcjZNOsYFTyqmeEo0tVZxS7KM56CpqnKrNMuEZpDbQhlT0tQIW1aVznVmweYpp8AYy9gYHa16u9C-LCD2snbRgPeqgXYRJc0FKUrGWTGgh2t0oWuwsguuVuFd_n1rAE5WwKvz8P6_p0T-aJCDBvmrQZ7dXv8G9g1rdH1K</recordid><startdate>201512</startdate><enddate>201512</enddate><creator>Sanders, Yvonne V.</creator><creator>Fijnvandraat, Karin</creator><creator>Boender, Johan</creator><creator>Mauser‐Bunschoten, Evelien P.</creator><creator>van der Bom, Johanna G.</creator><creator>de Meris, Joke</creator><creator>Smiers, Frans J.</creator><creator>Granzen, Bernd</creator><creator>Brons, Paul</creator><creator>Tamminga, Rienk Y.J.</creator><creator>Cnossen, Marjon H.</creator><creator>Leebeek, Frank W.G.</creator><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>7X8</scope></search><sort><creationdate>201512</creationdate><title>Bleeding spectrum in children with moderate or severe von Willebrand disease: Relevance of pediatric‐specific bleeding</title><author>Sanders, Yvonne V. ; Fijnvandraat, Karin ; Boender, Johan ; Mauser‐Bunschoten, Evelien P. ; van der Bom, Johanna G. ; de Meris, Joke ; Smiers, Frans J. ; Granzen, Bernd ; Brons, Paul ; Tamminga, Rienk Y.J. ; Cnossen, Marjon H. ; Leebeek, Frank W.G.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-p3565-f3a520b1dda5d04457eb1af7dab346b3e7d8acc912c6d9ffb7b4ded7251e33343</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2015</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Cross-Sectional Studies</topic><topic>Female</topic><topic>Hemorrhage - genetics</topic><topic>Humans</topic><topic>Male</topic><topic>von Willebrand Diseases - complications</topic><topic>von Willebrand Diseases - diagnosis</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Sanders, Yvonne V.</creatorcontrib><creatorcontrib>Fijnvandraat, Karin</creatorcontrib><creatorcontrib>Boender, Johan</creatorcontrib><creatorcontrib>Mauser‐Bunschoten, Evelien P.</creatorcontrib><creatorcontrib>van der Bom, Johanna G.</creatorcontrib><creatorcontrib>de Meris, Joke</creatorcontrib><creatorcontrib>Smiers, Frans J.</creatorcontrib><creatorcontrib>Granzen, Bernd</creatorcontrib><creatorcontrib>Brons, Paul</creatorcontrib><creatorcontrib>Tamminga, Rienk Y.J.</creatorcontrib><creatorcontrib>Cnossen, Marjon H.</creatorcontrib><creatorcontrib>Leebeek, Frank W.G.</creatorcontrib><creatorcontrib>WiN Study Group</creatorcontrib><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 hematology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Sanders, Yvonne V.</au><au>Fijnvandraat, Karin</au><au>Boender, Johan</au><au>Mauser‐Bunschoten, Evelien P.</au><au>van der Bom, Johanna G.</au><au>de Meris, Joke</au><au>Smiers, Frans J.</au><au>Granzen, Bernd</au><au>Brons, Paul</au><au>Tamminga, Rienk Y.J.</au><au>Cnossen, Marjon H.</au><au>Leebeek, Frank W.G.</au><aucorp>WiN Study Group</aucorp><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Bleeding spectrum in children with moderate or severe von Willebrand disease: Relevance of pediatric‐specific bleeding</atitle><jtitle>American journal of hematology</jtitle><addtitle>Am J Hematol</addtitle><date>2015-12</date><risdate>2015</risdate><volume>90</volume><issue>12</issue><spage>1142</spage><epage>1148</epage><pages>1142-1148</pages><issn>0361-8609</issn><eissn>1096-8652</eissn><abstract>The bleeding phenotype of children with von Willebrand disease (VWD) needs to be characterized in detail to facilitate diagnosis during childhood and aid in the planning and assessment of treatment strategies. 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Pediatric‐specific bleeding symptoms were present in 44% of patients. ISTH‐BAT bleeding score was higher in index cases than in affected family members (median, 12.0 vs. 6.5, P < 0.001), higher in Type 3 VWD than in Type 2 or 1 (17.0 vs. 10.5 or 6.5, P < 0.001) and higher in children with severe (<10 U/dL) than moderate VWD (10–30 U/dL) (11.0 vs. 7.0, P < 0.001). Frequency of any bleeding, epistaxis, and oral cavity was higher in types 2 and 3 than in Type 1 VWD and was associated with VWF levels. We conclude that pediatric‐specific bleeding symptoms occurred in a large proportion of children with moderate or severe VWD and should be included when evaluating children for VWD. Am. J. Hematol. 90:1142–1148, 2015. © 2015 Wiley Periodicals, Inc.</abstract><cop>United States</cop><pmid>26375306</pmid><doi>10.1002/ajh.24195</doi><tpages>7</tpages><oa>free_for_read</oa></addata></record>
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subjects	Adolescent Adult Child Child, Preschool Cross-Sectional Studies Female Hemorrhage - genetics Humans Male von Willebrand Diseases - complications von Willebrand Diseases - diagnosis Young Adult
title	Bleeding spectrum in children with moderate or severe von Willebrand disease: Relevance of pediatric‐specific bleeding
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