Acquired von Willebrand Disease Associated with Mantle Cell Lymphoma
Joint Authors
Maas, Dominique
Laros-van Gorkom, Britta
Gianotten, Sanne
Cruijsen, Marjan
van Heerde, Waander
Nijziel, Marten
Source
Issue
Vol. 2018, Issue 2018 (31 Dec. 2018), pp.1-3, 3 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2018-01-30
Country of Publication
Egypt
No. of Pages
3
Main Subjects
Abstract EN
We present a rare case of acquired von Willebrand syndrome (AVWS) caused by a mantle cell lymphoma.
A 61-year-old male suffered from recurrent bleeding symptoms since a few months.
Initially, physical examination was normal.
von Willebrand factor antigen (VWF:Ag) level and factor VIII activity (FVIII:C) were low (0.31 IU/ml and 0.43 IU/ml, resp.).
Ristocetin cofactor activity (VWF:RCo) was 0.09 IU/ml, and collagen binding activity (VWF:CB) was 0.10 IU/ml.
VWF:RCo/VWF:Ag ratio was 0.29, and RIPA value was normal.
Highest molecular weight VWF multimers were absent.
A diagnosis of von Willebrand Disease (VWD) type 2A was made.
However, no genetic mutation was found.
No inhibitory antibodies against VWF or factor VIII were detected.
A few months later, cervical, axillary, and inguinal lymphadenopathy was found on physical examination.
A CT scan confirmed multiple enlarged lymph nodes, and a clonal B-cell population matching a mantle cell lymphoma was detected in the bone marrow.
Chemoimmunotherapy resulted in a very good partial remission and concomitantly in a rapid decrease of bleeding problems and complete normalization of FVIII:C and VWF:Ag.
The diagnosis of AVWS cannot be rejected by negative mixing studies due to difficulties in the detection of autoantibodies and because of a highly heterogeneous pathogenesis of AVWS.
When the suspicion of AVWS is high, an extensive investigation should be performed to find the underlying cause.
American Psychological Association (APA)
Maas, Dominique& Laros-van Gorkom, Britta& Gianotten, Sanne& Cruijsen, Marjan& van Heerde, Waander& Nijziel, Marten. 2018. Acquired von Willebrand Disease Associated with Mantle Cell Lymphoma. Case Reports in Hematology،Vol. 2018, no. 2018, pp.1-3.
https://search.emarefa.net/detail/BIM-1144041
Modern Language Association (MLA)
Maas, Dominique…[et al.]. Acquired von Willebrand Disease Associated with Mantle Cell Lymphoma. Case Reports in Hematology No. 2018 (2018), pp.1-3.
https://search.emarefa.net/detail/BIM-1144041
American Medical Association (AMA)
Maas, Dominique& Laros-van Gorkom, Britta& Gianotten, Sanne& Cruijsen, Marjan& van Heerde, Waander& Nijziel, Marten. Acquired von Willebrand Disease Associated with Mantle Cell Lymphoma. Case Reports in Hematology. 2018. Vol. 2018, no. 2018, pp.1-3.
https://search.emarefa.net/detail/BIM-1144041
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1144041