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Open Journal of Hematology

ISSN: 2075-907X
Volume 8, 2017



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EMBASE

Open Journal of Hematology, 2014, 5-2 [Short Communication Research]

Rare Congenital Coagulation Factor Deficiencies: Clinical Manifestations

Yasmina Berkouk-Redjimi*, Meriem Fadela Belhani

Haematology Department, University Hospital Beni Messous, Issad Hassani, Algiers, Algeria

Corresponding Author & Address:

Yasmina Berkouk-Redjimi*
Haematology Department, University Hospital Beni Messous, Issad Hassani, Algiers, Algeria 16000. Tel/fax: +213 21931186; e-mail: redjimiberkouk@hotmail.com

Article History:
Published: 15th March, 2014   Accepted: 15th March, 2014
Received: 12th January, 2014      

© Berkouk-Redjimi et al.; licensee Ross Science Publishers

ROSS Open Access articles will be distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided that the original work will always be cited properly.

Abstract:

The clinical symptoms of rare bleeding disorders (RBDs) range from mucosal to life-threatening haemorrhages or functionally restricting haemorrhages. Congenital coagulation factor (F) deficiencies, VII, FX, FXIII, fibrinogen deficiencies and dysfibrinogenaemias, represent 3‒5% of all inherited coagulation factor deficiencies. Due to this low prevalence and the variability of bleeding manifestations, the clinical features of these RBDs are not well characterised. For this reason we conducted a retrospective multicentre national survey using patient records to characterize clinical bleeding manifestations in patients with congenital isolated/combined coagulation factor deficiencies or with congenital abnormalities of fibrinogen.

Fifteen departments (11 haematology and four paediatric departments) across Algeria took part in the study, and data from 234 patients were analyzed. The majority of patients (n=164; 70.1%) had isolated congenital coagulation factor deficiencies, the most common of which was FVII deficiency (n=101; 43.2%). Only 221 patients (9.0%) had combined congenital coagulation deficiencies, the most common of which was FVIII/V deficiency (n=13; 5.6%). Congenital fibrinogen abnormalities were reported in 49 patients (20.9%). Overall, there were 327 bleeding episodes in 163 patients. The majority of bleeding episodes (n=195, 59.6%) were in patients with an isolated factor deficiency, the highest incidence of which was recorded for FVII deficient patients (95 bleeds; mean 1.7 bleeds/patient).

Our results support existing descriptions in the literature and may help to target resourcing and plan treatment strategies for those patients at most risk of bleeding.



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