Acquired von Willebrand's disease due to aberrant expression of platelet glycoprotein Ib by marginal zone lymphoma cells

Ayalew Tefferi; Curtis A. Hanson; Paul J. Kurtin; Jerry A. Katzmann; Robert J. Dalton; William L. Nichols

doi:10.1046/j.1365-2141.1997.d01-2088.x

Acquired von Willebrand's disease due to aberrant expression of platelet glycoprotein Ib by marginal zone lymphoma cells

Ayalew Tefferi, Curtis A. Hanson, Paul J. Kurtin, Jerry A. Katzmann, Robert J. Dalton, William L. Nichols

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37 Scopus citations

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.

Original language	English (US)
Pages (from-to)	850-853
Number of pages	4
Journal	British journal of haematology
Volume	96
Issue number	4
DOIs	https://doi.org/10.1046/j.1365-2141.1997.d01-2088.x
State	Published - 1997

Keywords

acquired von Willebrand's disease
glycoprotein Ib
lymphoma

ASJC Scopus subject areas

Hematology

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10.1046/j.1365-2141.1997.d01-2088.x

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title = "Acquired von Willebrand's disease due to aberrant expression of platelet glycoprotein Ib by marginal zone lymphoma cells",

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.",

keywords = "acquired von Willebrand's disease, glycoprotein Ib, lymphoma",

author = "Ayalew Tefferi and Hanson, {Curtis A.} and Kurtin, {Paul J.} and Katzmann, {Jerry A.} and Dalton, {Robert J.} and Nichols, {William L.}",

year = "1997",

doi = "10.1046/j.1365-2141.1997.d01-2088.x",

language = "English (US)",

volume = "96",

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journal = "British journal of haematology",

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T1 - Acquired von Willebrand's disease due to aberrant expression of platelet glycoprotein Ib by marginal zone lymphoma cells

AU - Tefferi, Ayalew

AU - Hanson, Curtis A.

AU - Kurtin, Paul J.

AU - Katzmann, Jerry A.

AU - Dalton, Robert J.

AU - Nichols, William L.

PY - 1997

Y1 - 1997

N2 - 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.

AB - 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.

KW - acquired von Willebrand's disease

KW - glycoprotein Ib

KW - lymphoma

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DO - 10.1046/j.1365-2141.1997.d01-2088.x

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AN - SCOPUS:0031043222

SN - 0007-1048

VL - 96

SP - 850

EP - 853

JO - British journal of haematology

JF - British journal of haematology

IS - 4

ER -

Acquired von Willebrand's disease due to aberrant expression of platelet glycoprotein Ib by marginal zone lymphoma cells

Abstract

Keywords

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