Congenital Factor V Deficiency: Comparison of the Severity of Clinical Presentations among Patients with Rare Bleeding Disorders

Abstract

Background: Factor V deficiency (FVD) is a rare bleeding disorder (RBD) mostly present in regions with a high rate of consanguinity. FVD after FXIII deficiency is the next more prevalent RBD in Sistan and Baluchistan (S&B) in southeastern Iran. The aim of this study was to evaluate the clinical manifestations and severity of bleeding diathesis in patients with FVD. Methods: This descriptive study was conducted on 23 patients with FVD in S&B province. FVD was diagnosed by clinical findings and routine laboratory tests. Bleeding diatheses were classified into three grades (I-III) depending on the severity of symptoms. The severity of bleeding episodes in our patients was compared with other RBDs. Result: Based on residual plasma FV activity, 6 (26%), 16 (69.5%) and 1 (4.5%) patients had mild, moderate and severe factor deficiency, respectively. 24% of the patients had grade III life-threatening bleeding episodes which in comparison with FVII deficiency (17.4%) and FI deficiency (21%) had a higher incidence, and in comparison with FX deficiency (41.7%) and FXIII deficiency (63.1) had a lower incidence. Grade II and grade I bleeding diathesis were observed in 56.2 and 16.7% of the patients, respectively. Conclusion: FVD is the second most common type of RBD in S&B province and grade II bleeding episodes were the major bleeding presentation and observed in more than half of the patients.

Acquired factor V inhibitor: a nation‐wide study of 38 patients

“…Our study largely confirmed that AFVI occurs primarily in the elderly, which clearly differentiates this condition from hereditary forms of FV deficiency 38 . Accordingly, most patients suffered comorbidities including high blood pressure, diabetes mellitus and coronary artery disease, as previously reported 39 .…”

Bleeding manifestations in heterozygotes with congenital FVII deficiency: a comparison with unaffected family members during a long observation period

“…There is no possible comparison with FII or FV deficiency since no adequate study has been published so far. Sporadic data concerning FII or FV deficiency are nonconclusive [2,[29][30][31].…”

Diagnosis, clinical manifestations and management of rare bleeding disorders in Iran

“…Two of them had mild FV deficiency, while the other had moderate deficiency. FFP is used in the management of patients with FVD in Iran [17,28,31].…”

“…Most reported cases from southeast Iran had mild to moderate FVD, while most of the reported cases from central and northeastern Iran have moderate to severe deficiency [17,28,30].…”