Clinical and laboratory presentation of von Willebrand disease: Experience from a single center in Saudi Arabia.

Autor:	Owaidah T; Department of Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, KSA.; Department of Haematology and Transfusion Medicine, Alfaisal University, Riyadh, KSA., Alharbi M; Department of Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, KSA., Mandourah M; Department of Haematology and Transfusion Medicine, Alfaisal University, Riyadh, KSA., Saleh M; Department of Paediatric Haematology Oncology, King Faisal Specialist Hospital and Research Centre, Riyadh, KSA., Almusa A; Department of Paediatric Haematology Oncology, King Faisal Specialist Hospital and Research Centre, Riyadh, KSA., Alnounou R; Department of Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, KSA., Alzahrani H; Adult Hematology/HSCT, Oncology Centre, King Faisal Specialist Hospital and Research Center Riyadh, KSA., Khogeer H; Adult Hematology/HSCT, Oncology Centre, King Faisal Specialist Hospital and Research Center Riyadh, KSA.
Jazyk:	angličtina
Zdroj:	Journal of Taibah University Medical Sciences [J Taibah Univ Med Sci] 2022 Nov 14; Vol. 18 (2), pp. 413-419. Date of Electronic Publication: 2022 Nov 14 (Print Publication: 2023).
DOI:	10.1016/j.jtumed.2022.10.019
Abstrakt:	Objectives: This study was aimed at assessing the clinical presentations and laboratory findings among patients diagnosed with vWD at a Saudi tertiary care unit. Methods: This retrospective study included 189 patients with vWD who were followed up in our unit over 4 years. Clinical and laboratory data were collected and analyzed in SPSS. Results: The median age of the study cohort was 30 years (range 11 months-56 years). The cohort had a female preponderance, with 32.30% males and 66.70% females. Bleeding from different sites was observed, mostly from the joints and muscles (23.90%), followed by the mucus membranes (14.60%), genitourinary areas (7.70%), ecchymoses (2.80%), and gastrointestinal areas (2.80%). A total of 48% of participants presented with more than one type of bleeding. A total of 105 (58.01%) participants had type 1; 29 (16.02%) had type 2; and 47 (25.96%) had type 3 vWD. Blood tests indicated the following mean value: hemoglobin, 116 ± 25.60 gm/L; ferritin, 75.80 ± 166.80 μg/L (median 28.5); vWAg, 0.40 ± 0.27IU/ml; and vWD:RCo, 0.32 ± 0.20IU/dL. The partial thromboplastin time was prolonged in 49.20% and normal in 50.80% of participants. Platelet function analysis values were prolonged in 92.90% and normal in 7.10% of participants. Comparative analysis of the O-type and non-O blood type showed that blood type O was significantly correlated with factor VIII (p-value = 0.013), vWF:RCo (p-value = 0.004), and vWF:Ag (p-value = 0.019). Conclusion: Joint and muscle bleeds were the most common clinical presentations in our cohort. Although type 1 vWD was most prevalent in our cohort, we observed a comparatively higher prevalence of type 3, possibly because of ethnic differences or referral bias. We found a significant difference between O and non-O blood type regarding FVIII and vWF:Ag, and observed a more pronounced difference for vWD activity measuresd by vWF:RCo with blood type O being the systematic factor. (© 2022 [The Author/The Authors].)
Databáze:	MEDLINE
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