Acquired von Willebrand's disease due to aberrant expression of platelet glycoprotein Ib by marginal zone lymphoma cells
A 69‐year‐old woman presented with splenic marginal zone lymphoma associated with acquired von Willebrand's disease (AVWD). Laboratory abnormalities included markedly decreased plasma levels of factor VIII coagulant (C) activity (VIII:C 28%), von Willebrand’s factor (VWF) antigen (Ag) (vWF:Ag ...
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| Veröffentlicht in: | British journal of haematology 1997-03, Vol.96 (4), p.850-853 |
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| container_title | British journal of haematology |
| container_volume | 96 |
| creator | TEFFERI, AYALEW HANSON, CURTIS A. KURTIN, PAUL J. KATZMANN, JERRY A. DALTON, ROBERT J. NICHOLS, WILLIAM L. |
| description | A 69‐year‐old woman presented with splenic marginal zone lymphoma associated with acquired von Willebrand's disease (AVWD). Laboratory abnormalities included markedly decreased plasma levels of factor VIII coagulant (C) activity (VIII:C 28%), von Willebrand’s factor (VWF) antigen (Ag) (vWF:Ag |
| doi_str_mv | 10.1046/j.1365-2141.1997.d01-2088.x |
| format | Article |
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Laboratory abnormalities included markedly decreased plasma levels of factor VIII coagulant (C) activity (VIII:C 28%), von Willebrand’s factor (VWF) antigen (Ag) (vWF:Ag < 6%), and VWF ristocetin cofactor (RCo) activity (VWF:RCo, < 12%). VWF multimer analysis revealed a severe type II defect. Treatment with cryoprecipitate, high‐dose gamma globulin or desmopressin given intravenously was unsuccessful. Clinical bleeding and coagulation abnormalities showed transient improvement after replacement therapy with Humate‐P concentrate. The coagulation abnormalities improved partially after splenectomy and completely after subsequent chemotherapy. The neoplastic lymphocytes in the blood and spleen strongly expressed platelet glycoprotein Ib (CD42) and VWF but not other platelet‐associated antigens.</description><identifier>ISSN: 0007-1048</identifier><identifier>EISSN: 1365-2141</identifier><identifier>DOI: 10.1046/j.1365-2141.1997.d01-2088.x</identifier><identifier>PMID: 9074430</identifier><identifier>CODEN: BJHEAL</identifier><language>eng</language><publisher>Oxford, U.K. and Cambridge, USA: Blackwell Science Ltd</publisher><subject>acquired von Willebrand's disease ; Aged ; Antigens, Human Platelet - analysis ; Biological and medical sciences ; Female ; Flow Cytometry ; glycoprotein Ib ; Hematologic and hematopoietic diseases ; Humans ; Immunophenotyping ; Leukemia, Lymphocytic, Chronic, B-Cell - metabolism ; lymphoma ; Lymphoma, B-Cell - blood ; Lymphoma, B-Cell - complications ; Lymphoma, B-Cell - surgery ; Medical sciences ; Platelet diseases and coagulopathies ; Platelet Glycoprotein GPIb-IX Complex - metabolism ; Splenectomy ; Splenic Neoplasms - blood ; Splenic Neoplasms - complications ; Splenic Neoplasms - surgery ; von Willebrand Diseases - blood ; von Willebrand Diseases - etiology</subject><ispartof>British journal of haematology, 1997-03, Vol.96 (4), p.850-853</ispartof><rights>1997 INIST-CNRS</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c5209-c11bb00bab26988da0006019725f9a791719088ab83595b79e76668881259caa3</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1046%2Fj.1365-2141.1997.d01-2088.x$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1046%2Fj.1365-2141.1997.d01-2088.x$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>315,781,785,1418,1434,27929,27930,45579,45580,46414,46838</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=2591928$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/9074430$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>TEFFERI, AYALEW</creatorcontrib><creatorcontrib>HANSON, CURTIS A.</creatorcontrib><creatorcontrib>KURTIN, PAUL J.</creatorcontrib><creatorcontrib>KATZMANN, JERRY A.</creatorcontrib><creatorcontrib>DALTON, ROBERT J.</creatorcontrib><creatorcontrib>NICHOLS, WILLIAM L.</creatorcontrib><title>Acquired von Willebrand's disease due to aberrant expression of platelet glycoprotein Ib by marginal zone lymphoma cells</title><title>British journal of haematology</title><addtitle>Br J Haematol</addtitle><description>A 69‐year‐old woman presented with splenic marginal zone lymphoma associated with acquired von Willebrand's disease (AVWD). Laboratory abnormalities included markedly decreased plasma levels of factor VIII coagulant (C) activity (VIII:C 28%), von Willebrand’s factor (VWF) antigen (Ag) (vWF:Ag < 6%), and VWF ristocetin cofactor (RCo) activity (VWF:RCo, < 12%). VWF multimer analysis revealed a severe type II defect. Treatment with cryoprecipitate, high‐dose gamma globulin or desmopressin given intravenously was unsuccessful. Clinical bleeding and coagulation abnormalities showed transient improvement after replacement therapy with Humate‐P concentrate. The coagulation abnormalities improved partially after splenectomy and completely after subsequent chemotherapy. The neoplastic lymphocytes in the blood and spleen strongly expressed platelet glycoprotein Ib (CD42) and VWF but not other platelet‐associated antigens.</description><subject>acquired von Willebrand's disease</subject><subject>Aged</subject><subject>Antigens, Human Platelet - analysis</subject><subject>Biological and medical sciences</subject><subject>Female</subject><subject>Flow Cytometry</subject><subject>glycoprotein Ib</subject><subject>Hematologic and hematopoietic diseases</subject><subject>Humans</subject><subject>Immunophenotyping</subject><subject>Leukemia, Lymphocytic, Chronic, B-Cell - metabolism</subject><subject>lymphoma</subject><subject>Lymphoma, B-Cell - blood</subject><subject>Lymphoma, B-Cell - complications</subject><subject>Lymphoma, B-Cell - surgery</subject><subject>Medical sciences</subject><subject>Platelet diseases and coagulopathies</subject><subject>Platelet Glycoprotein GPIb-IX Complex - metabolism</subject><subject>Splenectomy</subject><subject>Splenic Neoplasms - blood</subject><subject>Splenic Neoplasms - complications</subject><subject>Splenic Neoplasms - surgery</subject><subject>von Willebrand Diseases - blood</subject><subject>von Willebrand Diseases - etiology</subject><issn>0007-1048</issn><issn>1365-2141</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1997</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqVkEFv1DAUhC1EVZbCT0CyBIJTwnOyiW1xQG0FtKgSFxBHy05eildOnNpJ2fDrcbSrvffkw8w8z3yEvGWQM9jWH3c5K-sqK9iW5UxKnrfAsgKEyPfPyOakPScbAOBZyogX5GWMOwBWQsXOybkEvt2WsCH7y-ZhtgFb-ugH-ts6hyboof0QaWsj6oi0nZFOnmqDISkTxf0YMEab_L6jo9MTOpzovVsaPwY_oR3oraFmob0O93bQjv7zA1K39OMf32vaoHPxFTnrtIv4-vhekF9fv_y8vsnufny7vb68y5qqAJk1jBkDYLQpailEq9OkGpjkRdVJzSXjTKbl2oiykpXhEnld10IIVlSy0bq8IO8Pd1O1hxnjpHob1wZ6QD9HxUViAaJOxk8HYxN8jAE7NQabFiyKgVq5q51a2aqVrVq5q8RdrdzVPqXfHL-ZTY_tKXsEnfR3R13HRrsukWxsPNlSWSYLkWyfD7a_1uHylAbq6vuNrGT5H8P1n-4</recordid><startdate>199703</startdate><enddate>199703</enddate><creator>TEFFERI, AYALEW</creator><creator>HANSON, CURTIS A.</creator><creator>KURTIN, PAUL J.</creator><creator>KATZMANN, JERRY A.</creator><creator>DALTON, ROBERT J.</creator><creator>NICHOLS, WILLIAM L.</creator><general>Blackwell Science Ltd</general><general>Blackwell</general><scope>IQODW</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>7X8</scope></search><sort><creationdate>199703</creationdate><title>Acquired von Willebrand's disease due to aberrant expression of platelet glycoprotein Ib by marginal zone lymphoma cells</title><author>TEFFERI, AYALEW ; HANSON, CURTIS A. ; KURTIN, PAUL J. ; KATZMANN, JERRY A. ; DALTON, ROBERT J. ; NICHOLS, WILLIAM L.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c5209-c11bb00bab26988da0006019725f9a791719088ab83595b79e76668881259caa3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1997</creationdate><topic>acquired von Willebrand's disease</topic><topic>Aged</topic><topic>Antigens, Human Platelet - analysis</topic><topic>Biological and medical sciences</topic><topic>Female</topic><topic>Flow Cytometry</topic><topic>glycoprotein Ib</topic><topic>Hematologic and hematopoietic diseases</topic><topic>Humans</topic><topic>Immunophenotyping</topic><topic>Leukemia, Lymphocytic, Chronic, B-Cell - metabolism</topic><topic>lymphoma</topic><topic>Lymphoma, B-Cell - blood</topic><topic>Lymphoma, B-Cell - complications</topic><topic>Lymphoma, B-Cell - surgery</topic><topic>Medical sciences</topic><topic>Platelet diseases and coagulopathies</topic><topic>Platelet Glycoprotein GPIb-IX Complex - metabolism</topic><topic>Splenectomy</topic><topic>Splenic Neoplasms - blood</topic><topic>Splenic Neoplasms - complications</topic><topic>Splenic Neoplasms - surgery</topic><topic>von Willebrand Diseases - blood</topic><topic>von Willebrand Diseases - etiology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>TEFFERI, AYALEW</creatorcontrib><creatorcontrib>HANSON, CURTIS A.</creatorcontrib><creatorcontrib>KURTIN, PAUL J.</creatorcontrib><creatorcontrib>KATZMANN, JERRY A.</creatorcontrib><creatorcontrib>DALTON, ROBERT J.</creatorcontrib><creatorcontrib>NICHOLS, WILLIAM L.</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>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>British journal of haematology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>TEFFERI, AYALEW</au><au>HANSON, CURTIS A.</au><au>KURTIN, PAUL J.</au><au>KATZMANN, JERRY A.</au><au>DALTON, ROBERT J.</au><au>NICHOLS, WILLIAM L.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Acquired von Willebrand's disease due to aberrant expression of platelet glycoprotein Ib by marginal zone lymphoma cells</atitle><jtitle>British journal of haematology</jtitle><addtitle>Br J Haematol</addtitle><date>1997-03</date><risdate>1997</risdate><volume>96</volume><issue>4</issue><spage>850</spage><epage>853</epage><pages>850-853</pages><issn>0007-1048</issn><eissn>1365-2141</eissn><coden>BJHEAL</coden><abstract>A 69‐year‐old woman presented with splenic marginal zone lymphoma associated with acquired von Willebrand's disease (AVWD). Laboratory abnormalities included markedly decreased plasma levels of factor VIII coagulant (C) activity (VIII:C 28%), von Willebrand’s factor (VWF) antigen (Ag) (vWF:Ag < 6%), and VWF ristocetin cofactor (RCo) activity (VWF:RCo, < 12%). VWF multimer analysis revealed a severe type II defect. Treatment with cryoprecipitate, high‐dose gamma globulin or desmopressin given intravenously was unsuccessful. Clinical bleeding and coagulation abnormalities showed transient improvement after replacement therapy with Humate‐P concentrate. The coagulation abnormalities improved partially after splenectomy and completely after subsequent chemotherapy. The neoplastic lymphocytes in the blood and spleen strongly expressed platelet glycoprotein Ib (CD42) and VWF but not other platelet‐associated antigens.</abstract><cop>Oxford, U.K. and Cambridge, USA</cop><pub>Blackwell Science Ltd</pub><pmid>9074430</pmid><doi>10.1046/j.1365-2141.1997.d01-2088.x</doi><tpages>4</tpages><oa>free_for_read</oa></addata></record> |
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| subjects | acquired von Willebrand's disease Aged Antigens, Human Platelet - analysis Biological and medical sciences Female Flow Cytometry glycoprotein Ib Hematologic and hematopoietic diseases Humans Immunophenotyping Leukemia, Lymphocytic, Chronic, B-Cell - metabolism lymphoma Lymphoma, B-Cell - blood Lymphoma, B-Cell - complications Lymphoma, B-Cell - surgery Medical sciences Platelet diseases and coagulopathies Platelet Glycoprotein GPIb-IX Complex - metabolism Splenectomy Splenic Neoplasms - blood Splenic Neoplasms - complications Splenic Neoplasms - surgery von Willebrand Diseases - blood von Willebrand Diseases - etiology |
| title | Acquired von Willebrand's disease due to aberrant expression of platelet glycoprotein Ib by marginal zone lymphoma cells |
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