Abstract Number: PB0786
Meeting: ISTH 2020 Congress
Background: Acquired Von Willebrand Syndrome (AVWS) is a rare acquired coagulopathy, often associated to an underlying disorder. Diagnosis is not easy and relies on a negative familial/personal hemorrhagic history and a late onset in life of bleedings, associated with a laboratory pattern for Von Willebrand Disease (VWD).
Aims: To describe the experience on diagnosis and management of AVWS patients (pts) in two Italian centers.
Methods: Diagnosis was made on the basis of clinical/laboratory features suggestive of AVWS. The screening for VWF:RCo inhibitor was made utilizing mixing studies. Data were collected from clinical charts.
Results: Between 2004-2019, 14 AVWS pts were diagnosed and managed [8F, 6M; median age 62.45 years (45.4-85.9)]. Reasons for diagnosis were: recent onset of bleeding symptoms in 9 pts, increased aPTT in 5. Patients characteristics are reported in table 1. All the pts did not have either a family or past personal history of hemorrhages. The spontaneous bleeding events presented at diagnosis and during follow up were: GI and gum bleeding, muscle hematomas, hematuria, ecchymoses, epistaxis, menometrorrhagia. Three pts bled after dental extractions/procedures, although they were prophylactically treated with VWF/FVIII concentrate. Desmopressin, Tranexamic Acid, Intravenous Immunoglobulins (IvIg) VWF/FVIII concentrate, and recombinant FVIII were used as prophylaxis or treatment of bleedings in all pts, with variable responses on clinical symptoms. Thirteen/14 cases showed a concomitant disorder: 1 gastric B cell MALT lymphoma, 1 indolent B cell lymphoma, 7 MGUS, 2 Waldenstrom Disease and 2 patient with breast cancer and concomitant MGUS. In one case AVWS was idiopathic. Patients management is reported in table 2.
Conclusions: AVWS is a rare syndrome, probably underdiagnosed because unrecognized. AVWS must be suspected whenever a patient presents a late onset in life of bleeding symptoms associated to laboratory characteristics compatible with VWD. It is mandatory to search for concomitant diseases.
|AVWS type 1, 2 A, 3||3 patients, 7 patients, 4 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%)|
|VWF:RCo inhibitor (searched in 4 cases)||3 negative, 1 positive|
|Gastric B cell MALT lymphoma||Rituximab||Complete remission of lymphoma and AVWS|
|Waldenstrom Disease (1st patient)||R-CVP, Ibrutinib||After 2nd line therapy, stable lymphoproliferative disease and persistent AVWS|
|Waldenstrom Disease (2nd patient||Rituximab. After two years for disease progression: Rituximab+Bendamustine||Partial response after Rituximab. Persistent AVWS and partial response of lymphoma after Rituximab+Bendamustine|
|1 Indolent B cell lymphoma +3 MGUS||No treatment||NA|
|MGUS 1 patient||Prednisone + cyclophosphamide and then IvIg||No response to either therapies on AVWS|
|MGUS 3 patients||Infusion of IvIg (2 cases are under chronic treatment with Iv Ig every 6-8 weeks)||Transient response on AVWS|
|1 patient with breast cancer + MGUS||Breast surgery||No response on AVWS|
|1 patient with breast cancer + MGUS||Prednisone||No response on AVWS Improvement of bleeding symptoms|
|Idiopathic AVWS||Prednisone. At relapse Prednisone + Cyclophosphamide||CR on AVWS after first line therapy and at relapse|
To cite this abstract in AMA style:Ferretti A, Baldacci E, Basso M, Abbruzzese R, De Luca ML, Barone F, Bochicchio R, Chistolini A, Mazzucconi MG, De Candia E, Santoro C. Diagnosis and Management of Acquired Von Willebrand Disease in Two Italian Centers [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/diagnosis-and-management-of-acquired-von-willebrand-disease-in-two-italian-centers/. Accessed January 28, 2022.
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