Abstract Number: OC 13.2
Meeting: ISTH 2021 Congress
Background: Gastro-intestinal (GI) bleeding is a characteristic feature of Von Willebrand disease (VWD), that arises generally in older patients, requiring in most cases blood transfusion and hospitalization. The presence of arteriovenous malformations is often described. Patients with congenital type 3, 2A and 2B are those most frequently affected by this symptom, which could be due to the loss of high-molecular-weight multimers (HMWM).
Aims: To evaluate the response to treatment in case of GI bleeding in patients with VWD.
Methods: All charts from patients with VWD evaluated at the A. Bianchi Bonomi Hemophilia and Thrombosis Center in Milan between 2009 and 2019 were analyzed. GI bleeding was defined as occult (positive stool test) or evident (hematemesis or melena).
Results: After exclusion of 13 type 3 carriers, 4 not classified patients and 2 patients with a bleeding comorbidity (Idiopathic Thrombocytopenia and Hereditary hemorrhagic telangiectasia), a total of 293 patients were evaluated. Forty-seven patients (16%) had at least one episode of GI bleeding during their life (occult in 4) and in 13 of them angiodysplastic lesions were documented at least on one occasion. Blood transfusions were required in 66% (31/47 patients). Endoscopic evaluation found a local lesion (ulcer, gastric or intestinal polyps or mucosal inflammation) in 38% of patients (18/47). Recurrent bleeding was present in 57% of patients (27/47). Tables 1 and 2 present patients and GI bleeding characteristics.
|Table 1||All patients, N=293||Patients with GI bleeding, N=47|
|Female sex, Number (%)||182 (62%)||25 (53%)|
|Age at diagnosis, years, (median, IQR)||22 (11-39)||36 (18-47)|
|Age at follow-up, years, (median, IQR)||45 (29-61)||66 (49-81)|
|FVIII:C, IU/dL (median, IQR)||46 (33-64)||31 (18-48)|
|VWF:RCo, IU/dL (median, IQR)||13 (3-33)||5 (3-12)|
|VWD with loss of HMWM (type 3, severe type1, 2A and 2B), Number (%)||118 (40%)||32 (68%)|
|VWD without loss of HMWM (type 2M, type 1, low levels of VWF, not classified), Number (%)||175 (60%)||15 (32%)|
|VWD with loss of HMWM (type 3, 2A and 2B), median age at follow-up (IQR)||45 (24-58)||60 (37-83)|
|VWD without loss of HMWM (type 2M type 1, low levels of VWF, not classified), median age at follow-up (IQR)||46 (31-63)||72 (64-81)|
|Table 2||Patients with GI bleeding, N=47||VWD with loss of HMWM, N=32||VWD without loss of HMWM, N=15|
|Age at first GI bleeding, years, (median, IQR)||46 (24-68)||44 (21-62)||53 (28-76)|
|GI bleeding event before diagnosis, Number (%)||19 (40%)||12||7|
|Local lesion causing GI bleeding, Number (%)||18 (38%)||7||11|
|Recurrent GI bleeding, Number (%)||27 (57%)||19||8|
|Presence of angiodysplasia, Number (%)||13 (28%)||11||2|
|No treatment with FVIII/VWF concentrate, Number (%)||21 (45%)||12||9|
|Treatment (FVIII/VWF concentrate) with complete response, Number (%)||17 (35%)||12||5|
|Long term prophylaxis (FVIII/VWF concentrate) with complete response, Number (%)||4 (9%)||4||0|
|Need of rescue therapy associated with prophylaxis, Number (%)||5 (11%)||4||1|
Conclusions: Of the 293 patients affected by VWD, followed at the A. Bianchi Bonomi Hemophilia and Thromobosis Center in Milano, 47 (16%) had at least 1 episode of GI bleeding during their life. Nine patients with GI bleeding (n=9, 19%) needed long term prophylaxis or some rescue therapy (surgery, atorvastatin, octreotide, lenalinomide) and 8 of them were characterized by loss of HMWM.
To cite this abstract in AMA style:Biguzzi E, Siboni SM, Braham S, Pagliari MT, Peyvandi F. Response to Treatment for Gastro-intestinal Bleeding in Patients Affected by von Willebrand Disease [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 1). https://abstracts.isth.org/abstract/response-to-treatment-for-gastro-intestinal-bleeding-in-patients-affected-by-von-willebrand-disease/. Accessed September 24, 2021.
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