ABSTRACT
To examine real-life clinical data regarding hereditary factor XI (FXI) deficiency from a secondary care centre. Retrospective review of clinical records for every FXI:C 0.7âIU/ml or less reported from 2012 to 2020. Seventy-nine patients were included. Six (7.6%) had a severe deficiency (FXI:C <0.2âIU/ml). Only 55 (69.6%) patients were referred to the Haemostasis Centre. Among them, six (15%) were subsequently not identified at increased haemorrhagic risk before a surgical/obstetrical procedure. Thirty-three (41.8%) experienced at least one bleeding event, minor (25 patients) and/or major (16 patients). Minor bleedings were predominantly spontaneous and more frequent in women, major events were mainly provoked. No correlation was found between FXI:C and risk of bleeding ( P â=â0.9153). Lower FXI:C, but not a positive bleeding history, was related with higher likelihood of being referred to the Haemostasis Centre ( P â=â0.0333). Hereditary FXI deficiency prevalence is likely underestimated, real-life clinical practices outside reference centres could be suboptimal.
Subject(s)
Factor XI Deficiency , Factor XI , Female , Humans , Factor XI/genetics , Factor XI Deficiency/epidemiology , Factor XI Deficiency/genetics , Hemorrhage/complications , Italy/epidemiology , Neglected Diseases/complications , Retrospective Studies , MaleABSTRACT
Laryngeal adult-type hemangiomas are very rare lesions, more frequent in men, whose optimal treatment consists of microlaryngoscopical excision. We herein report a case of larynx cavernous hemangioma in a 64-year-old woman with hoarseness for about six months. Histologically, the tumor was composed of multiple vessels embedded in an edematous stroma.