Single-Center Experience of von Willebrand Disease (vWD) Among Patients with Menorrhagia: A Diagnosis which could be Missed

Congenital factor deficiencies (CFDs) refer to inherited deficiency of coagulation factors in the blood. A total of 481 patients with CFDs, who were diagnosed and followed at our Pediatric Hematology and Oncology Clinic between 1990 and 2015, were retrospectively evaluated. Of the 481 cases, 134 (27.8%) were hemophilia A, 38 (7.9%) were hemophilia B, 57 (11.8%) were von Willebrand disease (vWD), and 252 (52.3%) were rare bleeding disorders (RBDs). The median age of the patients at the time of diagnosis and at the time of the study was 4.1 years (range: 2 months to 20.4 years) and 13.4 years (range: 7 months to 31.3 years), respectively. The median duration of the follow-up time was 6.8 years (range: 2.5 months to 24.8 years). One hundred nineteen (47.2%) of 252 patients with RBDs were asymptomatic, 49 (41.1%) of whom diagnosed by family histories, 65 (54.6%) through preoperative laboratory studies, and 5 (4.2%) after prolonged bleeding during surgeries. Consanguinity rate for the RBDs was 47.2%. Prophylactic treatment was initiated in 80 patients, 58 of whom were hemophilia A, 7 were hemophilia B, 13 were RBDs, and 2 were vWD. Significant advances have been achieved during the past 2 decades in the treatment of patients with CFDs, particularly in patients with hemophilias. The rarity and clinical heterogeneity of RBDs lead to significant diagnostic challenges and improper management. In this regard, multinational collaborative efforts are needed with the hope that can improve the management of patients with RBDs.

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von Willebrand Disease in Children: Diagnosis and Management of a Pediatric Cohort in One Single Center in Argentina

Seminars in Thrombosis and Hemostasis ◽

10.1055/s-0031-1281043 ◽

2011 ◽

Vol 37 (05) ◽

pp. 560-567 ◽

Cited By ~ 2

Author(s):

Mariana Bonduel ◽

Juan Frontroth ◽

Mirta Hepner ◽

Gabriela Sciuccati ◽

Aurora Feliu-Torres ◽

...

Keyword(s):

Von Willebrand Disease ◽

Single Center ◽

Diagnosis And Management ◽

Von Willebrand

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Dental invasive procedures in von Willebrand disease outpatients treated with high purity FVIII/VWF complex concentrate (Fanhdi®): experience of a single center

Heliyon ◽

10.1016/j.heliyon.2020.e03426 ◽

2020 ◽

Vol 6 (2) ◽

pp. e03426 ◽

Cited By ~ 1

Author(s):

Valeria De Padua ◽

Umberto Romeo ◽

Cristina Santoro ◽

Riccardo Bosco ◽

Erminia Baldacci ◽

...

Keyword(s):

High Purity ◽

Von Willebrand Disease ◽

Single Center ◽

Invasive Procedures ◽

Von Willebrand

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Acquired von Willebrand disease in chronic myeloproliferative disorders: a prospective single-center study

Medical Science and Discovery ◽

10.17546/msd.584596 ◽

2019 ◽

pp. 123-127

Author(s):

Ömer Ekinci ◽

Muhammed Aslanboğa

Keyword(s):

Myeloproliferative Disorders ◽

Von Willebrand Disease ◽

Single Center ◽

Chronic Myeloproliferative Disorders ◽

Acquired Von Willebrand Disease ◽

Single Center Study ◽

Von Willebrand ◽

Center Study

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Acquired von Willebrand disease: from theory to practice. A single center experience - three case reports

Revista Romana de Medicina de Laborator ◽

10.1515/rrlm-2016-0005 ◽

2016 ◽

Vol 24 (1) ◽

pp. 93-102

Author(s):

Andrei Colită ◽

Carmen Saguna ◽

Andra Costache ◽

Gabriela Borsaru ◽

Raluca Manolache ◽

...

Keyword(s):

Case Reports ◽

Von Willebrand Disease ◽

Hospital Department ◽

Single Center Experience ◽

Acquired Von Willebrand Disease ◽

History Of ◽

Underlying Diseases ◽

Bleeding History ◽

Von Willebrand ◽

Willebrand Factor

Abstract Acquired von Willebrand disease (AvWD) represents a rare, potentially severe and most likely underdiagnosed category of hemorrhagic syndromes determined by quantitative, qualitative or functional, nonhereditary, alterations of von Willebrand factor (vWF) that occur in the context of various underlying diseases. It is diagnosed mainly in adults, without any personal or familial history of bleeding. The etiopathogeny of AvWD is complex, marked by the intervention of multiple mechanisms, occuring in the evolution of neoplasia, autoimmune disorders, cardiovascular diseases and other conditions. The clinical and laboratory manifestations are similar to the congenital form with mucocutaneous hemorrhage in patients without bleeding history and demonstration of quantitative and/or functional anomalies of vWF. Treatment has two major objectives: control of bleeding and therapy of the underlying condition. As a practical illustration of the theoretical aspects we present 3 clinical cases of AvWD diagnosed in the Colţea Hospital Department of Hematology during the last 10 years.

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Orthopedic approach of haemophiliacs

Hämostaseologie ◽

10.1055/s-0037-1617117 ◽

2008 ◽

Vol 28 (S 01) ◽

pp. S52-S54

Author(s):

M.-D. Mihailov ◽

D. Poenaru ◽

L. Pop ◽

I. Branea ◽

M. Bataneant ◽

...

Keyword(s):

Retrospective Analysis ◽

Hemophilia A ◽

Von Willebrand Disease ◽

Single Center ◽

Orthopedic Treatment ◽

Clinical Expression ◽

Demand Substitution ◽

Non Invasive ◽

On Demand ◽

Von Willebrand

SummaryClinical expression of inadequately treated haemophilia is dominated by orthopedic complications, requiring invasive or non-invasive interventions. Objective: In Romania, with under dosed and late introduced “on demand” substitution, we aimed at highlighting the experience of orthopedic treatment and its outcome. Patients, methods: Single center retrospective analysis regarding orthopedic interventions and their outcomes was conducted on 59 hemophilia A, B, and von Willebrand disease patients, between 2002 and 2007. Results: The majority of interventions, invasive (60.71%) and non-invasive (39.28%), were elective, only two being emergencies. Postoperative functional evolution after synovectomies was good in 68.28%, fair in 24.39%, satisfactory in 7.31%. Results of 33 non-invasive (extensive releases) procedures were very good in 27.27%, good in 63.63%, poor in 9.09%. Discussion, conclusions: The important number and complexity of orthopedic interventions are proving the precarious musculoskeletal state in persons with hemophilia, demonstrating the need of improving substitution, at least with discontinue prophylaxis in patients with severe forms.

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