Two Rare Complications in One Patient: Acquired von Willebrand Syndrome Associated with Intracranial Plasmacytoma

Here, we describe a rare case of acquired von Willebrand syndrome (VWS) associated with intracranial plasmacytoma. The literature includes reports of a few cases of plasmacytoma with central nervous involvement, but none of them with acquired VWS. Diagnosis was made based on a stereotaxic intracereb...

Full description

Saved in:
Bibliographic Details
Main Authors: H. Auge, C. Yguel, E. Schmitt, B. Frotscher, H. Busby-Venner, R. Morizot, C. Moulin, P. Feugier, A. Perrot, L. Filliatre-Clement
Format: Article
Language:English
Published: Wiley 2019-01-01
Series:Case Reports in Hematology
Online Access:http://dx.doi.org/10.1155/2019/7609308
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1832554829241647104
author H. Auge
C. Yguel
E. Schmitt
B. Frotscher
H. Busby-Venner
R. Morizot
C. Moulin
P. Feugier
A. Perrot
L. Filliatre-Clement
author_facet H. Auge
C. Yguel
E. Schmitt
B. Frotscher
H. Busby-Venner
R. Morizot
C. Moulin
P. Feugier
A. Perrot
L. Filliatre-Clement
author_sort H. Auge
collection DOAJ
description Here, we describe a rare case of acquired von Willebrand syndrome (VWS) associated with intracranial plasmacytoma. The literature includes reports of a few cases of plasmacytoma with central nervous involvement, but none of them with acquired VWS. Diagnosis was made based on a stereotaxic intracerebral biopsy. During this biopsy, a ventriculoperitoneal shunt was established, which was complicated with abnormal bleeding. Subsequent hemostasis assessment related to hemopathy revealed acquired von Willebrand disease. The patient received induction therapy with bortezomib, thalidomide, and dexamethasone (VTD), followed by high-dose melphalan chemotherapy and autologous stem cell transplantation, and then VTD consolidation, and finally maintenance with lenalidomide. Our patient currently remains in very good partial response without neurological symptoms after 4 months of maintenance. The patient is free of progression 14 months after their original presentation.
format Article
id doaj-art-39793381a795495e953642bc5aa5a2b0
institution Kabale University
issn 2090-6560
2090-6579
language English
publishDate 2019-01-01
publisher Wiley
record_format Article
series Case Reports in Hematology
spelling doaj-art-39793381a795495e953642bc5aa5a2b02025-02-03T05:50:24ZengWileyCase Reports in Hematology2090-65602090-65792019-01-01201910.1155/2019/76093087609308Two Rare Complications in One Patient: Acquired von Willebrand Syndrome Associated with Intracranial PlasmacytomaH. Auge0C. Yguel1E. Schmitt2B. Frotscher3H. Busby-Venner4R. Morizot5C. Moulin6P. Feugier7A. Perrot8L. Filliatre-Clement9Hematology Department, University Hospital of Nancy, 5 Rue du Morvan, 54500 Vandoeuvre les Nancy, FranceHistopathology Department, University Hospital of Nancy, 5 Rue du Morvan, 54500 Vandoeuvre les Nancy, FranceNeuroradiology Department, University Hospital of Nancy, 29 Avenue du Maréchal de Lattre de Tassigny, 54035 Nancy, FranceHemostasis Department, University Hospital of Nancy, 5 Rue du Morvan, 54500 Vandoeuvre les Nancy, FranceHistopathology Department, University Hospital of Nancy, 5 Rue du Morvan, 54500 Vandoeuvre les Nancy, FranceHematology Department, University Hospital of Nancy, 5 Rue du Morvan, 54500 Vandoeuvre les Nancy, FranceHematology Department, University Hospital of Nancy, 5 Rue du Morvan, 54500 Vandoeuvre les Nancy, FranceHematology Department, University Hospital of Nancy, 5 Rue du Morvan, 54500 Vandoeuvre les Nancy, FranceHematology Department, University Hospital of Nancy, 5 Rue du Morvan, 54500 Vandoeuvre les Nancy, FranceHematology Department, University Hospital of Nancy, 5 Rue du Morvan, 54500 Vandoeuvre les Nancy, FranceHere, we describe a rare case of acquired von Willebrand syndrome (VWS) associated with intracranial plasmacytoma. The literature includes reports of a few cases of plasmacytoma with central nervous involvement, but none of them with acquired VWS. Diagnosis was made based on a stereotaxic intracerebral biopsy. During this biopsy, a ventriculoperitoneal shunt was established, which was complicated with abnormal bleeding. Subsequent hemostasis assessment related to hemopathy revealed acquired von Willebrand disease. The patient received induction therapy with bortezomib, thalidomide, and dexamethasone (VTD), followed by high-dose melphalan chemotherapy and autologous stem cell transplantation, and then VTD consolidation, and finally maintenance with lenalidomide. Our patient currently remains in very good partial response without neurological symptoms after 4 months of maintenance. The patient is free of progression 14 months after their original presentation.http://dx.doi.org/10.1155/2019/7609308
spellingShingle H. Auge
C. Yguel
E. Schmitt
B. Frotscher
H. Busby-Venner
R. Morizot
C. Moulin
P. Feugier
A. Perrot
L. Filliatre-Clement
Two Rare Complications in One Patient: Acquired von Willebrand Syndrome Associated with Intracranial Plasmacytoma
Case Reports in Hematology
title Two Rare Complications in One Patient: Acquired von Willebrand Syndrome Associated with Intracranial Plasmacytoma
title_full Two Rare Complications in One Patient: Acquired von Willebrand Syndrome Associated with Intracranial Plasmacytoma
title_fullStr Two Rare Complications in One Patient: Acquired von Willebrand Syndrome Associated with Intracranial Plasmacytoma
title_full_unstemmed Two Rare Complications in One Patient: Acquired von Willebrand Syndrome Associated with Intracranial Plasmacytoma
title_short Two Rare Complications in One Patient: Acquired von Willebrand Syndrome Associated with Intracranial Plasmacytoma
title_sort two rare complications in one patient acquired von willebrand syndrome associated with intracranial plasmacytoma
url http://dx.doi.org/10.1155/2019/7609308
work_keys_str_mv AT hauge tworarecomplicationsinonepatientacquiredvonwillebrandsyndromeassociatedwithintracranialplasmacytoma
AT cyguel tworarecomplicationsinonepatientacquiredvonwillebrandsyndromeassociatedwithintracranialplasmacytoma
AT eschmitt tworarecomplicationsinonepatientacquiredvonwillebrandsyndromeassociatedwithintracranialplasmacytoma
AT bfrotscher tworarecomplicationsinonepatientacquiredvonwillebrandsyndromeassociatedwithintracranialplasmacytoma
AT hbusbyvenner tworarecomplicationsinonepatientacquiredvonwillebrandsyndromeassociatedwithintracranialplasmacytoma
AT rmorizot tworarecomplicationsinonepatientacquiredvonwillebrandsyndromeassociatedwithintracranialplasmacytoma
AT cmoulin tworarecomplicationsinonepatientacquiredvonwillebrandsyndromeassociatedwithintracranialplasmacytoma
AT pfeugier tworarecomplicationsinonepatientacquiredvonwillebrandsyndromeassociatedwithintracranialplasmacytoma
AT aperrot tworarecomplicationsinonepatientacquiredvonwillebrandsyndromeassociatedwithintracranialplasmacytoma
AT lfilliatreclement tworarecomplicationsinonepatientacquiredvonwillebrandsyndromeassociatedwithintracranialplasmacytoma