Decreased clearance of von Willebrand factor in a patient with type 2B von Willebrand disease following development of immune thrombocytopenia
We report a case of concurrent type 2B von Willebrand disease (VWD) and immune thrombocytopenia (ITP). The patient had characteristic loss of von Willebrand factor (VWF) high molecular weight multimers (HMWM) but a normal platelet count in the initial 8 years after diagnosis of type 2B VWD. When he...
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| Veröffentlicht in: | Pediatric Blood & Cancer 2008-09, Vol.51 (3), p.416-418 |
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| container_title | Pediatric Blood & Cancer |
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| creator | Wang, Zhihong J. Onwuzurike, Nkechi Callaghan, Michael U. Rajpurkar, Madhvi Chitlur, Meera Lusher, Jeanne M. |
| description | We report a case of concurrent type 2B von Willebrand disease (VWD) and immune thrombocytopenia (ITP). The patient had characteristic loss of von Willebrand factor (VWF) high molecular weight multimers (HMWM) but a normal platelet count in the initial 8 years after diagnosis of type 2B VWD. When he developed severe thrombocytopenia, however, both his VWD indices and VWF HMWM normalized. As his platelet count increased, he again lost the HMWM and his VWD indices decreased. These results suggest that the severe thrombocytopenia led to decreased clearance of VWF, especially the HMWM. Pediatr Blood Cancer 2008;51:416–418. © 2008 Wiley‐Liss, Inc. |
| doi_str_mv | 10.1002/pbc.21593 |
| format | Article |
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The patient had characteristic loss of von Willebrand factor (VWF) high molecular weight multimers (HMWM) but a normal platelet count in the initial 8 years after diagnosis of type 2B VWD. When he developed severe thrombocytopenia, however, both his VWD indices and VWF HMWM normalized. As his platelet count increased, he again lost the HMWM and his VWD indices decreased. These results suggest that the severe thrombocytopenia led to decreased clearance of VWF, especially the HMWM. 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Blood Cancer</addtitle><description>We report a case of concurrent type 2B von Willebrand disease (VWD) and immune thrombocytopenia (ITP). The patient had characteristic loss of von Willebrand factor (VWF) high molecular weight multimers (HMWM) but a normal platelet count in the initial 8 years after diagnosis of type 2B VWD. When he developed severe thrombocytopenia, however, both his VWD indices and VWF HMWM normalized. As his platelet count increased, he again lost the HMWM and his VWD indices decreased. These results suggest that the severe thrombocytopenia led to decreased clearance of VWF, especially the HMWM. Pediatr Blood Cancer 2008;51:416–418. © 2008 Wiley‐Liss, Inc.</description><subject>Adolescent</subject><subject>Dimerization</subject><subject>hemostasis</subject><subject>Humans</subject><subject>ITP</subject><subject>Male</subject><subject>Platelet Count</subject><subject>thrombocytopenia</subject><subject>Thrombocytopenia - complications</subject><subject>Thrombocytopenia - diagnosis</subject><subject>Thrombocytopenia - immunology</subject><subject>von Willebrand Diseases - complications</subject><subject>von Willebrand Factor - analysis</subject><subject>VWD</subject><subject>VWF</subject><issn>1545-5009</issn><issn>1545-5017</issn><issn>1096-911X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2008</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkc1u1DAURi1ERX9gwQsgr5C6SGvHcRIvmfRXqgoL0Cwtx7mmBicOtmem8xJ95mY60yIhVax8ZZ1zNh9CHyk5oYTkp2OrT3LKBXuDDigveMYJrd6-3ETso8MYf01oSXj9Du3TuhBMiOoAPZyBDqAidFg7UEENGrA3eOkHPLfOQTt9ddgonXzAdsAKjypZGBJe2XSH03oEnM_-5TsbN1FsvHN-ZYefuIMlOD_2G3Pq275fDIDTXfB96_U6-REGq96jPaNchA-79wj9uDj_3lxlN18vr5svN5ku8opl2pAyp0VOVMsYZ0obUXPatlwYTo3Qnag0q2ipSiJKzjSvDam56YhRQhStYUfo87Y7Bv9nATHJ3kYNzqkB_CLKUrDJr-l_wZyUVNRFNYHHW1AHH2MAI8dgexXWkhK5WUlOK8mnlSb20y66aHvo_pK7WSbgdAusrIP16yX5bdY8J7OtYWOC-xdDhd-yrFjF5fz2Us5nFw1pKJMNewQCd6za</recordid><startdate>200809</startdate><enddate>200809</enddate><creator>Wang, Zhihong J.</creator><creator>Onwuzurike, Nkechi</creator><creator>Callaghan, Michael U.</creator><creator>Rajpurkar, Madhvi</creator><creator>Chitlur, Meera</creator><creator>Lusher, Jeanne M.</creator><general>Wiley Subscription Services, Inc., A Wiley Company</general><scope>BSCLL</scope><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>7T5</scope><scope>H94</scope><scope>7X8</scope></search><sort><creationdate>200809</creationdate><title>Decreased clearance of von Willebrand factor in a patient with type 2B von Willebrand disease following development of immune thrombocytopenia</title><author>Wang, Zhihong J. ; Onwuzurike, Nkechi ; Callaghan, Michael U. ; Rajpurkar, Madhvi ; Chitlur, Meera ; Lusher, Jeanne M.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4273-cf0621420ab3353acf9851bb59f51f9cd97c3716a609653c58f085fd0fa994bf3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2008</creationdate><topic>Adolescent</topic><topic>Dimerization</topic><topic>hemostasis</topic><topic>Humans</topic><topic>ITP</topic><topic>Male</topic><topic>Platelet Count</topic><topic>thrombocytopenia</topic><topic>Thrombocytopenia - complications</topic><topic>Thrombocytopenia - diagnosis</topic><topic>Thrombocytopenia - immunology</topic><topic>von Willebrand Diseases - complications</topic><topic>von Willebrand Factor - analysis</topic><topic>VWD</topic><topic>VWF</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Wang, Zhihong J.</creatorcontrib><creatorcontrib>Onwuzurike, Nkechi</creatorcontrib><creatorcontrib>Callaghan, Michael U.</creatorcontrib><creatorcontrib>Rajpurkar, Madhvi</creatorcontrib><creatorcontrib>Chitlur, Meera</creatorcontrib><creatorcontrib>Lusher, Jeanne M.</creatorcontrib><collection>Istex</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>Pediatric Blood & Cancer</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Wang, Zhihong J.</au><au>Onwuzurike, Nkechi</au><au>Callaghan, Michael U.</au><au>Rajpurkar, Madhvi</au><au>Chitlur, Meera</au><au>Lusher, Jeanne M.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Decreased clearance of von Willebrand factor in a patient with type 2B von Willebrand disease following development of immune thrombocytopenia</atitle><jtitle>Pediatric Blood & Cancer</jtitle><addtitle>Pediatr. Blood Cancer</addtitle><date>2008-09</date><risdate>2008</risdate><volume>51</volume><issue>3</issue><spage>416</spage><epage>418</epage><pages>416-418</pages><issn>1545-5009</issn><eissn>1545-5017</eissn><eissn>1096-911X</eissn><abstract>We report a case of concurrent type 2B von Willebrand disease (VWD) and immune thrombocytopenia (ITP). The patient had characteristic loss of von Willebrand factor (VWF) high molecular weight multimers (HMWM) but a normal platelet count in the initial 8 years after diagnosis of type 2B VWD. When he developed severe thrombocytopenia, however, both his VWD indices and VWF HMWM normalized. As his platelet count increased, he again lost the HMWM and his VWD indices decreased. These results suggest that the severe thrombocytopenia led to decreased clearance of VWF, especially the HMWM. 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| ispartof | Pediatric Blood & Cancer, 2008-09, Vol.51 (3), p.416-418 |
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| language | eng |
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| source | Wiley-Blackwell Journals; MEDLINE |
| subjects | Adolescent Dimerization hemostasis Humans ITP Male Platelet Count thrombocytopenia Thrombocytopenia - complications Thrombocytopenia - diagnosis Thrombocytopenia - immunology von Willebrand Diseases - complications von Willebrand Factor - analysis VWD VWF |
| title | Decreased clearance of von Willebrand factor in a patient with type 2B von Willebrand disease following development of immune thrombocytopenia |
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