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Acquired von Willebrand Disease: The Diagnosis and Management of an Underdiagnosed Coagulopathy

A. Ferretti1, E. Baldacci1, S. Lancellotti2, M. Basso2, F. Barone2, A. Pallotta3, G. Lapietra1, M. Sacco2, A. Chistolini1, E. De Candia2, C. Santoro1

1Unit of Hematology, Department of Translational and Precision Medicine, Sapienza- University of Rome, Rome, Italy, 2Fondazione Policlinico Gemelli IRCCS, Università Cattolica Sacro Cuore, Rome, Italy, 3Unit of Hematology, Department of Translational and Precision Medicine, Rome, Italy

Abstract Number: PB0938

Meeting: ISTH 2021 Congress

Theme: Platelet Disorders, von Willebrand Disease and Thrombotic Microangiopathies » VWF and von Willebrand Factor Disorders - Clinical Conditions

Background: Acquired Von Willebrand Syndrome (AVWS) is an acquired coagulopathy, often associated to an underlying disorder. The diagnosis is not easy and relies on a negative familial and personal clinical hemorrhagic history and a late onset in life of bleeding symptoms, associated with a laboratory pattern for Von Willebrand Disease (VWD).

Aims: Aim of this study is to describe the experience on diagnosis and management of AVWS patients (pts) in two Italian centers.

Methods: Between 2004-2020 we have diagnosed and managed 14 pts [8F, 6M; median age 62.45 years (45.4-85.9)] affected by AVWS. Determination of coagulation parameters, including FVIII, were carried out on an automatic coagulometer (ACL Top 700, Werfen). VWF:antigen (VWF:Ag) and VWF:activity (VWF:RCo) were measured by chemiluminescence assays (HemosIL AcuStar, Werfen). VWF propeptide (VWFpp) level was measured by ELISA immunoassay (Sanquin, Amsterdam, NL). The screening for VWF:RCo inhibitor was made utilizing mixing studies

Results: At diagnosis, for all pts, we observed the results showed in table 1. VWFpp and multimers were studied just in 9 pts. Except VWFpp median level, all other VWF-related median parameters were significantly reduced. VWFpp/VWF:Ag ratio was significantly higher in all 9 studied pts; multimers were absent in 6 and normal in 2; in 1 patient just LMW multimers were present. All the pts did not have either a family or past personal history of bleeding symptoms. Thirteen/14 cases showed a concomitant disorder, one case was idiopathic.  In table 2, therapies either for underlying disorders if present or AVWS, and outcomes are shown.

Table 1. Clinical and laboratory characteristics of patients
Median VWF:Ag
(n.v. blood group 0: 41-101%; no 0: 50-130%)
15%
(range 1.6-51%)
 
Median VWF:RCo
(n.v. blood group 0: 41-97%; no 0: 52-124)
 
13%
(range <6.25-33%)
Median FVIII:C
(n.v.58-130%)
19.2%
(range 2.1-53%)
Median VWFpp
(n.v. 70-140)
81.5 %
range 42.9-154.3
Median VWFpp/VWF:Ag
(n.v. ratio <3.0)
20.0
VWF:RCo inhibitor (searched in 4 cases) 3 negative, 1 positive

Clinical and laboratory characteristics of patients

Table 2. Management of the patients with lymphoproliferative disorders
 
Type Treatment Response
Gastric B cell MALT lymphoma
1 patient
Rituximab Complete remission of lymphoma and AVWS
Waldenstrom Disease
1st patient
R-CVP
Ibrutinib
After 2nd line, stable lymphoproliferative disease and persistent AVWS
Waldenstrom Disease
2nd patient
Rituximab
 
After two years for disease progression: Rituximab+-Bendamustine
Partial response
 
Persistent AVWS and partial response of lymphoma
Indolent B cell lymphoma
1 patient
Watch and wait strategy
 
Rituximab
 
 
 
 
Complete remission of lymphoma and AVWS
Strategies to manage AVWS in all  patients
Type  of disorder Treatment Response
MGUS
1 patient
Prednisone + cyclophosphamide and then high dose immunoglobulins No response to either therapies
MGUS
3 patients
Infusion of high dose immunoglobulins
(2 cases are under chronic treatment with Iv Ig every 6-8 weeks)
Transient CR as regard VWD laboratory parameters
MGUS
4 patients
No treatment
 
 
Breast cancer
1 patient 
Prednisone Response to bleeding’s symptoms
Idiopathic AVWS
1 patients
Prednisone
 
At relapse Prednisone + cyclophosphamide 
CR on VWD laboratory parameters
 
CR on VWD laboratory parameters

Management of the patients with lymphoproliferative disorders

Conclusions: AVWS is a rare syndrome, probably underdiagnosed. AVWS must be suspected whenever a patient presents with a late onset in life of bleeding symptoms associated to laboratory characteristics compatible with VWD. The VWFpp/VWF:Ag ratio could be a valuable biomarker to be considered to help the diagnosis.

To cite this abstract in AMA style:

Ferretti A, Baldacci E, Lancellotti S, Basso M, Barone F, Pallotta A, Lapietra G, Sacco M, Chistolini A, De Candia E, Santoro C. Acquired von Willebrand Disease: The Diagnosis and Management of an Underdiagnosed Coagulopathy [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/acquired-von-willebrand-disease-the-diagnosis-and-management-of-an-underdiagnosed-coagulopathy/. Accessed November 30, 2023.

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