Different bleeding risk in type 2A and 2M von Willebrand disease: a 2‐year prospective study in 107 patients
Background: Type 2A and 2M von Willebrand disease (VWD2A and VWD2M) are characterized by the presence of a dysfunctional von Willebrand factor (VWF) and a variable bleeding tendency. So far, a head‐to‐head comparison of the clinical history and bleeding risk between VWD2A and VWD2M has never been p...
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| Veröffentlicht in: | Journal of thrombosis and haemostasis 2012-04, Vol.10 (4), p.632-638 |
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| creator | CASTAMAN, G. FEDERICI, A. B. TOSETTO, A. LA MARCA, S. STUFANO, F. MANNUCCI, P. M. RODEGHIERO, F. |
| description | Background: Type 2A and 2M von Willebrand disease (VWD2A and VWD2M) are characterized by the presence of a dysfunctional von Willebrand factor (VWF) and a variable bleeding tendency. So far, a head‐to‐head comparison of the clinical history and bleeding risk between VWD2A and VWD2M has never been provided in a prospective manner.
Aim of the study: We assessed the bleeding incidence rate and clinical characteristics in two cohorts of 17 families (46 patients) with VWD2A and 15 families (61 patients) with VWD2M prospectively followed‐up for 24 months. VWF gene mutations were characterized in all of them.
Results: Mean bleeding score (BS) and VWF antigen at enrollment were significantly higher in VWD2A patients (P = 0.007). No correlation between VWF activity or factor VIII levels and the severity of BS was observed. The incidence rate of spontaneous bleeding requiring treatment was 107/100 patient‐years (95% CI, 88.3–131) in VWD2A compared with 40/100 patient‐years (95% CI, 30–53) in VWD2M (P |
| doi_str_mv | 10.1111/j.1538-7836.2012.04661.x |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_1014100059</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>1014100059</sourcerecordid><originalsourceid>FETCH-LOGICAL-c4021-baa5bfda09398bdfeb1251de5c4acfe6d2ae618b4fc2eb18a1504b6285b1522a3</originalsourceid><addsrcrecordid>eNqNkcFO3DAQhq2qqFDKKyAfuWzqmcRZB4nDaoFuEYgLFUfLjifISzabxlnY3HiEPmOfpE4XOOOLRzPf_GPPzxgHkUA835cJyFRNpirNExSAicjyHJLtJ3bwXvj8Fhdpus--hrAUAgqJ4gvbR0yxmBZ4wJpzX1XUUdNzWxM53zzwzodH7hveDy1xnHHTOI43_Gnd8Htf12S7MeN8IBPolBuOf1_-DGQ63nbr0FLZ-yfiod-4YZQBMeWt6X2cEb6xvcrUgY5e70P26_Libr6YXN_--DmfXU_KTCBMrDHSVs6IIi2UdRVZQAmOZJmZsqLcoaEclM2qEmNNGZAiszkqaUEimvSQnex044t-byj0euVDSXVtGlpvggYBGQghZPERFFSWCyUiqnZoGf8ZOqp02_mV6YYI6dEYvdTjzvW4fz0ao_8bo7ex9fh1ysauyL03vjkRgbMd8OxrGj4srK_uFmOU_gMBH5yD</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>1011846080</pqid></control><display><type>article</type><title>Different bleeding risk in type 2A and 2M von Willebrand disease: a 2‐year prospective study in 107 patients</title><source>MEDLINE</source><source>Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals</source><source>Alma/SFX Local Collection</source><creator>CASTAMAN, G. ; FEDERICI, A. B. ; TOSETTO, A. ; LA MARCA, S. ; STUFANO, F. ; MANNUCCI, P. M. ; RODEGHIERO, F.</creator><creatorcontrib>CASTAMAN, G. ; FEDERICI, A. B. ; TOSETTO, A. ; LA MARCA, S. ; STUFANO, F. ; MANNUCCI, P. M. ; RODEGHIERO, F.</creatorcontrib><description>Background: Type 2A and 2M von Willebrand disease (VWD2A and VWD2M) are characterized by the presence of a dysfunctional von Willebrand factor (VWF) and a variable bleeding tendency. So far, a head‐to‐head comparison of the clinical history and bleeding risk between VWD2A and VWD2M has never been provided in a prospective manner.
Aim of the study: We assessed the bleeding incidence rate and clinical characteristics in two cohorts of 17 families (46 patients) with VWD2A and 15 families (61 patients) with VWD2M prospectively followed‐up for 24 months. VWF gene mutations were characterized in all of them.
Results: Mean bleeding score (BS) and VWF antigen at enrollment were significantly higher in VWD2A patients (P = 0.007). No correlation between VWF activity or factor VIII levels and the severity of BS was observed. The incidence rate of spontaneous bleeding requiring treatment was 107/100 patient‐years (95% CI, 88.3–131) in VWD2A compared with 40/100 patient‐years (95% CI, 30–53) in VWD2M (P < 0.001). The risk of bleeding was significantly higher in patients with BS ≥ 10 at enrollment compared with those with BS 0–2. Furthermore, 54 episodes of gastrointestinal bleeding occurred in 17/46 (36.9%) VWD2A patients and seven in 2/61 (3.3%) VWD2M patients (P < 0.0001).
Conclusion: Bleeding tendency in VWD2A is greater than that of VWD2M, is not explained by factor VIII or VWF levels and is mainly due to an increased incidence of gastrointestinal bleeding.</description><identifier>ISSN: 1538-7933</identifier><identifier>ISSN: 1538-7836</identifier><identifier>EISSN: 1538-7836</identifier><identifier>DOI: 10.1111/j.1538-7836.2012.04661.x</identifier><identifier>PMID: 22329792</identifier><language>eng</language><publisher>Oxford, UK: Blackwell Publishing Ltd</publisher><subject>Adolescent ; Adult ; Aged ; Aged, 80 and over ; Factor VIII - analysis ; Female ; Gastrointestinal Hemorrhage - etiology ; gene mutation ; Genetic Predisposition to Disease ; Hemorrhage - epidemiology ; Hemorrhage - etiology ; Hemorrhage - genetics ; Hemorrhage - therapy ; Hemostatic Techniques ; Historical account ; Humans ; Incidence ; inherited bleeding disorder ; Italy - epidemiology ; Male ; Middle Aged ; Multivariate Analysis ; Mutation ; Phenotype ; Prognosis ; Proportional Hazards Models ; Prospective Studies ; Risk Assessment ; Risk Factors ; thromboembolism ; Time Factors ; von Willebrand disease ; von Willebrand Disease, Type 2 - complications ; von Willebrand Disease, Type 2 - epidemiology ; von Willebrand Disease, Type 2 - genetics ; von Willebrand factor ; von Willebrand Factor - analysis ; von Willebrand Factor - genetics ; Young Adult</subject><ispartof>Journal of thrombosis and haemostasis, 2012-04, Vol.10 (4), p.632-638</ispartof><rights>2012 International Society on Thrombosis and Haemostasis</rights><rights>2012 International Society on Thrombosis and Haemostasis.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4021-baa5bfda09398bdfeb1251de5c4acfe6d2ae618b4fc2eb18a1504b6285b1522a3</citedby><cites>FETCH-LOGICAL-c4021-baa5bfda09398bdfeb1251de5c4acfe6d2ae618b4fc2eb18a1504b6285b1522a3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,27901,27902</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/22329792$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>CASTAMAN, G.</creatorcontrib><creatorcontrib>FEDERICI, A. B.</creatorcontrib><creatorcontrib>TOSETTO, A.</creatorcontrib><creatorcontrib>LA MARCA, S.</creatorcontrib><creatorcontrib>STUFANO, F.</creatorcontrib><creatorcontrib>MANNUCCI, P. M.</creatorcontrib><creatorcontrib>RODEGHIERO, F.</creatorcontrib><title>Different bleeding risk in type 2A and 2M von Willebrand disease: a 2‐year prospective study in 107 patients</title><title>Journal of thrombosis and haemostasis</title><addtitle>J Thromb Haemost</addtitle><description>Background: Type 2A and 2M von Willebrand disease (VWD2A and VWD2M) are characterized by the presence of a dysfunctional von Willebrand factor (VWF) and a variable bleeding tendency. So far, a head‐to‐head comparison of the clinical history and bleeding risk between VWD2A and VWD2M has never been provided in a prospective manner.
Aim of the study: We assessed the bleeding incidence rate and clinical characteristics in two cohorts of 17 families (46 patients) with VWD2A and 15 families (61 patients) with VWD2M prospectively followed‐up for 24 months. VWF gene mutations were characterized in all of them.
Results: Mean bleeding score (BS) and VWF antigen at enrollment were significantly higher in VWD2A patients (P = 0.007). No correlation between VWF activity or factor VIII levels and the severity of BS was observed. The incidence rate of spontaneous bleeding requiring treatment was 107/100 patient‐years (95% CI, 88.3–131) in VWD2A compared with 40/100 patient‐years (95% CI, 30–53) in VWD2M (P < 0.001). The risk of bleeding was significantly higher in patients with BS ≥ 10 at enrollment compared with those with BS 0–2. Furthermore, 54 episodes of gastrointestinal bleeding occurred in 17/46 (36.9%) VWD2A patients and seven in 2/61 (3.3%) VWD2M patients (P < 0.0001).
Conclusion: Bleeding tendency in VWD2A is greater than that of VWD2M, is not explained by factor VIII or VWF levels and is mainly due to an increased incidence of gastrointestinal bleeding.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Aged</subject><subject>Aged, 80 and over</subject><subject>Factor VIII - analysis</subject><subject>Female</subject><subject>Gastrointestinal Hemorrhage - etiology</subject><subject>gene mutation</subject><subject>Genetic Predisposition to Disease</subject><subject>Hemorrhage - epidemiology</subject><subject>Hemorrhage - etiology</subject><subject>Hemorrhage - genetics</subject><subject>Hemorrhage - therapy</subject><subject>Hemostatic Techniques</subject><subject>Historical account</subject><subject>Humans</subject><subject>Incidence</subject><subject>inherited bleeding disorder</subject><subject>Italy - epidemiology</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Multivariate Analysis</subject><subject>Mutation</subject><subject>Phenotype</subject><subject>Prognosis</subject><subject>Proportional Hazards Models</subject><subject>Prospective Studies</subject><subject>Risk Assessment</subject><subject>Risk Factors</subject><subject>thromboembolism</subject><subject>Time Factors</subject><subject>von Willebrand disease</subject><subject>von Willebrand Disease, Type 2 - complications</subject><subject>von Willebrand Disease, Type 2 - epidemiology</subject><subject>von Willebrand Disease, Type 2 - genetics</subject><subject>von Willebrand factor</subject><subject>von Willebrand Factor - analysis</subject><subject>von Willebrand Factor - genetics</subject><subject>Young Adult</subject><issn>1538-7933</issn><issn>1538-7836</issn><issn>1538-7836</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2012</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNkcFO3DAQhq2qqFDKKyAfuWzqmcRZB4nDaoFuEYgLFUfLjifISzabxlnY3HiEPmOfpE4XOOOLRzPf_GPPzxgHkUA835cJyFRNpirNExSAicjyHJLtJ3bwXvj8Fhdpus--hrAUAgqJ4gvbR0yxmBZ4wJpzX1XUUdNzWxM53zzwzodH7hveDy1xnHHTOI43_Gnd8Htf12S7MeN8IBPolBuOf1_-DGQ63nbr0FLZ-yfiod-4YZQBMeWt6X2cEb6xvcrUgY5e70P26_Libr6YXN_--DmfXU_KTCBMrDHSVs6IIi2UdRVZQAmOZJmZsqLcoaEclM2qEmNNGZAiszkqaUEimvSQnex044t-byj0euVDSXVtGlpvggYBGQghZPERFFSWCyUiqnZoGf8ZOqp02_mV6YYI6dEYvdTjzvW4fz0ao_8bo7ex9fh1ysauyL03vjkRgbMd8OxrGj4srK_uFmOU_gMBH5yD</recordid><startdate>201204</startdate><enddate>201204</enddate><creator>CASTAMAN, G.</creator><creator>FEDERICI, A. B.</creator><creator>TOSETTO, A.</creator><creator>LA MARCA, S.</creator><creator>STUFANO, F.</creator><creator>MANNUCCI, P. M.</creator><creator>RODEGHIERO, F.</creator><general>Blackwell Publishing Ltd</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>7X8</scope><scope>7U1</scope><scope>7U2</scope><scope>C1K</scope></search><sort><creationdate>201204</creationdate><title>Different bleeding risk in type 2A and 2M von Willebrand disease: a 2‐year prospective study in 107 patients</title><author>CASTAMAN, G. ; FEDERICI, A. B. ; TOSETTO, A. ; LA MARCA, S. ; STUFANO, F. ; MANNUCCI, P. M. ; RODEGHIERO, F.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4021-baa5bfda09398bdfeb1251de5c4acfe6d2ae618b4fc2eb18a1504b6285b1522a3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2012</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Aged</topic><topic>Aged, 80 and over</topic><topic>Factor VIII - analysis</topic><topic>Female</topic><topic>Gastrointestinal Hemorrhage - etiology</topic><topic>gene mutation</topic><topic>Genetic Predisposition to Disease</topic><topic>Hemorrhage - epidemiology</topic><topic>Hemorrhage - etiology</topic><topic>Hemorrhage - genetics</topic><topic>Hemorrhage - therapy</topic><topic>Hemostatic Techniques</topic><topic>Historical account</topic><topic>Humans</topic><topic>Incidence</topic><topic>inherited bleeding disorder</topic><topic>Italy - epidemiology</topic><topic>Male</topic><topic>Middle Aged</topic><topic>Multivariate Analysis</topic><topic>Mutation</topic><topic>Phenotype</topic><topic>Prognosis</topic><topic>Proportional Hazards Models</topic><topic>Prospective Studies</topic><topic>Risk Assessment</topic><topic>Risk Factors</topic><topic>thromboembolism</topic><topic>Time Factors</topic><topic>von Willebrand disease</topic><topic>von Willebrand Disease, Type 2 - complications</topic><topic>von Willebrand Disease, Type 2 - epidemiology</topic><topic>von Willebrand Disease, Type 2 - genetics</topic><topic>von Willebrand factor</topic><topic>von Willebrand Factor - analysis</topic><topic>von Willebrand Factor - genetics</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>CASTAMAN, G.</creatorcontrib><creatorcontrib>FEDERICI, A. B.</creatorcontrib><creatorcontrib>TOSETTO, A.</creatorcontrib><creatorcontrib>LA MARCA, S.</creatorcontrib><creatorcontrib>STUFANO, F.</creatorcontrib><creatorcontrib>MANNUCCI, P. M.</creatorcontrib><creatorcontrib>RODEGHIERO, F.</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><collection>Risk Abstracts</collection><collection>Safety Science and Risk</collection><collection>Environmental Sciences and Pollution Management</collection><jtitle>Journal of thrombosis and haemostasis</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>CASTAMAN, G.</au><au>FEDERICI, A. B.</au><au>TOSETTO, A.</au><au>LA MARCA, S.</au><au>STUFANO, F.</au><au>MANNUCCI, P. M.</au><au>RODEGHIERO, F.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Different bleeding risk in type 2A and 2M von Willebrand disease: a 2‐year prospective study in 107 patients</atitle><jtitle>Journal of thrombosis and haemostasis</jtitle><addtitle>J Thromb Haemost</addtitle><date>2012-04</date><risdate>2012</risdate><volume>10</volume><issue>4</issue><spage>632</spage><epage>638</epage><pages>632-638</pages><issn>1538-7933</issn><issn>1538-7836</issn><eissn>1538-7836</eissn><abstract>Background: Type 2A and 2M von Willebrand disease (VWD2A and VWD2M) are characterized by the presence of a dysfunctional von Willebrand factor (VWF) and a variable bleeding tendency. So far, a head‐to‐head comparison of the clinical history and bleeding risk between VWD2A and VWD2M has never been provided in a prospective manner.
Aim of the study: We assessed the bleeding incidence rate and clinical characteristics in two cohorts of 17 families (46 patients) with VWD2A and 15 families (61 patients) with VWD2M prospectively followed‐up for 24 months. VWF gene mutations were characterized in all of them.
Results: Mean bleeding score (BS) and VWF antigen at enrollment were significantly higher in VWD2A patients (P = 0.007). No correlation between VWF activity or factor VIII levels and the severity of BS was observed. The incidence rate of spontaneous bleeding requiring treatment was 107/100 patient‐years (95% CI, 88.3–131) in VWD2A compared with 40/100 patient‐years (95% CI, 30–53) in VWD2M (P < 0.001). The risk of bleeding was significantly higher in patients with BS ≥ 10 at enrollment compared with those with BS 0–2. Furthermore, 54 episodes of gastrointestinal bleeding occurred in 17/46 (36.9%) VWD2A patients and seven in 2/61 (3.3%) VWD2M patients (P < 0.0001).
Conclusion: Bleeding tendency in VWD2A is greater than that of VWD2M, is not explained by factor VIII or VWF levels and is mainly due to an increased incidence of gastrointestinal bleeding.</abstract><cop>Oxford, UK</cop><pub>Blackwell Publishing Ltd</pub><pmid>22329792</pmid><doi>10.1111/j.1538-7836.2012.04661.x</doi><tpages>7</tpages></addata></record> |
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| subjects | Adolescent Adult Aged Aged, 80 and over Factor VIII - analysis Female Gastrointestinal Hemorrhage - etiology gene mutation Genetic Predisposition to Disease Hemorrhage - epidemiology Hemorrhage - etiology Hemorrhage - genetics Hemorrhage - therapy Hemostatic Techniques Historical account Humans Incidence inherited bleeding disorder Italy - epidemiology Male Middle Aged Multivariate Analysis Mutation Phenotype Prognosis Proportional Hazards Models Prospective Studies Risk Assessment Risk Factors thromboembolism Time Factors von Willebrand disease von Willebrand Disease, Type 2 - complications von Willebrand Disease, Type 2 - epidemiology von Willebrand Disease, Type 2 - genetics von Willebrand factor von Willebrand Factor - analysis von Willebrand Factor - genetics Young Adult |
| title | Different bleeding risk in type 2A and 2M von Willebrand disease: a 2‐year prospective study in 107 patients |
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