VPB0839 - Diagnostic Errors Of Von Willebrand Disease In A Developing Country: Analysis Of 38 Years Data

Monday, July 11, 2022

6:30 PM – 7:30 PM

Location: Exhibition

Presentation Format: Virtual

Presenting Author(s)

LH

Liana Hambardzumyan

Co-Author(s)

NM

Nare Martirosyan

Hematologist
Hematology Center after Prof. R. H. Yeolyan, Yerevan, Armenia
Yerevan, Yerevan, Armenia
AM

Alisa Movsisyan

Resident
Hematology Center after Prof. R.H. Yeolyan, Yerevan, Armenia, Department of Pediatric Oncology and Hematology, Yerevan State Medical University, Yerevan, Armenia,
Yerevan, Yerevan, Armenia
MB

Maria Badikyan

Hematologist
Hematology Center after Prof. R.H. Yeolyan
Yerevan, Yerevan, Armenia
NS

Nelli S. Sargsyan

MD
Armenian Hemophilia and Thrombosis Treatment center
Yerevan, Yerevan, Armenia
GT

Gevorg N. Tamamyan

Head of the Department of Pediatric Oncology and Hematology, Yerevan State Medical University
Armenian Hemophilia and Thrombosis Treatment center
Yerevan, Yerevan, Armenia
Anahit Ter-Grigoryan

Head of Center
Armenian Hemophilia and Thrombosis Center Yerevan State Medical University, Department of Pediatric Oncology and Hematology
Yerevan, Yerevan, Armenia

Background: Von Willebrand disease (VWD) is the most common inherited bleeding disorder. The incidence of symptomatic cases is 1 in 10,000 to 1 in 100,000.

Aims: The aim of our study is to evaluate the diagnostic workup and management of VWD in Armenia.

Methods: The medical records of all patients with VWD being managed in the only hematology center in Armenia (Hematology Center after Prof. R.H.Yeolyan), were retrospectively reviewed and analyzed.

Results: Since 1984 VWD was diagnosed in 32 patients (~1 in 100,000 of Armenia population) with the median age of 30 and mean age of 31.2 years. Four of them were falsely diagnosed with and managed as platelet-type VWD by immunophenotyping. Sixteen patients were female (50%). Six (35.3%) of 17 available blood typing testes were O+. Two patients had no bleeding history, easy bruising and mucocutaneous bleeding occurred in 30 patients (93.8%), 10 (31.3%) of them had also joint, soft tissue bleeding, 2 had gastrointestinal and 7 (43.8% of females) had heavy menstrual bleeding. Anemia developed in 43.8% of cases. Fourteen patients (43.8%) had family history of bleeding. Von Willebrand factor antigen and functional assays (VWF:Ag and/or VWF:Act) were performed in 17 patients: 6 were < 30%, 1 was 30-50% and 10 were > 50% (3 of them had decreased FVIII/VWF:Ag), multimers weren't assessed. FVIII activity was measured in 24 and was decreased in 14 (58.3%) cases. Automated analyzer revealed thrombocytopenia in 9 patients and blood film morphology in 3 of them. Nineteen (59,4%) patients had prolonged APTT. Antifibrinolytics were used in all bleeding episodes, after major bleeding 13 patients received plasma-derived VWF concentrate, 3 patients FVIII, 1 patient desmopressin acetate.

Conclusion(s): VWD screening tests aren't constantly available and completely performed in Armenia so there is a high probability of misdiagnosis. Further efforts are needed for rediagnosis, subtype classifying and differential diagnosis.