Successful use of lenalidomide to treat refractory acquired von Willebrand disease associated with monoclonal gammopathy.
Ann Hematol
; 101(12): 2627-2631, 2022 Dec.
Article
in English
| MEDLINE | ID: covidwho-2261233
ABSTRACT
Acquired von Willebrand syndrome (AVWS) is a rare hematologic disorder characterized by quantitative or qualitative defects of von Willebrand factor (vWF), a protein crucial for normal hemostasis. AVWS has been described in association with several pathologic entities with varied mechanisms. Among these, lymphoproliferative disorders are the most common, with monoclonal gammopathy of undetermined significance (MGUS) being the most frequently reported. AVWS in this setting is commonly associated with the development of bleeding that is clinically challenging to manage due to accelerated clearance of vWF, limiting the utility of many conventional treatment modalities such as DDAVP or vWF/FVIII. We report a case of a 43-year-old male who was sent to our institution for new-onset easy bruising and laboratories concerning for von Willebrand disease (vWD). Further diagnostic workup revealed evidence of an IgG monoclonal gammopathy and findings suggestive of vWF inhibition. Ultimately, he was found to have monoclonal gammopathy of clinical significance (MGCS)-associated AVWS refractory to conventional treatment but responsive to lenalidomide and dexamethasone. This case suggests that lenalidomide may be suitable for patients with AVWS secondary to MGCS.
Keywords
Full text:
Available
Collection:
International databases
Database:
MEDLINE
Main subject:
Paraproteinemias
/
Von Willebrand Diseases
/
Monoclonal Gammopathy of Undetermined Significance
Type of study:
Case report
/
Diagnostic study
/
Prognostic study
/
Qualitative research
Topics:
Long Covid
Limits:
Adult
/
Humans
/
Male
Language:
English
Journal:
Ann Hematol
Journal subject:
Hematology
Year:
2022
Document Type:
Article
Affiliation country:
S00277-022-04991-9
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