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. 2023 May 24;9(6):e16376.
doi: 10.1016/j.heliyon.2023.e16376. eCollection 2023 Jun.

Survival analysis of coagulation disorders: A retrospective study with a 5-year follow-up

Arman Jahangiri  1 , Sara Ahmadi  2 , Hassan Rafieemehr  3
Affiliations

Survival analysis of coagulation disorders: A retrospective study with a 5-year follow-up

Arman Jahangiri et al. Heliyon. .

Abstract

Objective: Rare bleeding disorders (RBDs) are the diseases in which patients experience a deficiency of coagulation factors. In the management of these disorders, surveillance is a significant challenge. This study aimed to assess the survival of patients with RBDs in a five-year follow-up.

Materials and methods: This descriptive cross-sectional study was conducted on 146 patients with RBDs who had referred to Be'sat Hospital of Hamadan, Iran from July 2017 to August 2022. A computerized record search was performed to identify the patients. The surveillance time for a five-year follow-up was assessed with the Kaplan-Meier curve. A log-rank test also served to compare the survival rates according to the type of factor.

Results: Out of 146 patients, 117 (80.2%) were males and 29 (19.8%) were females. They were in the range of 2-59 years of age with a mean of 23.11 ± 14.6. The most common disorder was FVIII deficiency (65.8%), and the rarest one was FXIII deficiency (4.8%). The rate of survival for any reason was 54.42 ± 1.3 months. The survival in combined FV and FVIII deficiencies was found to be longer than in the other deficiencies (55.9 ± 5.7), but there was no significant difference (P ≥ 0.05). In contrast, the survival in FXIII deficiency was observed to be lower than the other cases (44 ± 9.6); however, no significant difference was found in this regard (P ≥ 0.05).

Conclusion: The results of this study show that patients with RBDs have different rates of survival, which suggests that identifying high-risk patients may be helpful for the improvement of their survival time through timely therapeutic interventions.

Keywords: Bleeding disorders; Blood coagulation; Hemophilia; Survival.

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Conflict of interest statement

The authors have no interests to declare.

Figures

Fig. 1
Fig. 1
The 5-year survival of patients with coagulation disorders.
Fig. 2
Fig. 2
The 5-year survival of patients with combined FV and FVIII deficiency in comparison with other RBDs: The average survival of patients with combined FV and FVIII deficiency was 55.9 months. For the other RBD cases, it was 54.3 months. Based on the results of the log-rank test, there was no statistically significant difference of survival time between the two groups of patients (P = 0.993).
Fig. 3
Fig. 3
The 5-year survival of patients with FXIII deficiency in comparison with the other RBD patients: The average survival of patients with FXIII deficiency was 44 ± 9.6 months. For the other RBD cases, it was 54.9 ± 1.2 months. Based on the results of the log-rank test, there was no statistically significant difference of survival time between the two groups of patients (P = 0.112). (+) indicates FXIII censoring.
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References

    1. O'donnell J.S., O'sullivan J.M., Preston R.J. Advances in understanding the molecular mechanisms that maintain normal haemostasis. Br. J. Haematol. 2019;186:24–36. doi: 10.1111/bjh.15872. - DOI - PubMed
    1. Palla R., Peyvandi F., Shapiro A.D. Rare bleeding disorders: diagnosis and treatment. Blood. 2015;125(13):2052–2061. doi: 10.1182/blood-2014-08-532820. - DOI - PubMed
    1. Jain S., Acharya S.S. Rare coagulation factor deficiencies. Hem. Adol. Fem. 2020;57(6):51–60. doi: 10.1016/j.transci.2018.10.009. - DOI
    1. Batsuli G., Kouides P. Rare coagulation factor deficiencies (Factors VII, X, V, and II) Hematol./Oncol. Clin. 2021;35(6):1181–1196. doi: 10.1016/j.hoc.2021.07.010. - DOI - PubMed
    1. Menegatti M., Peyvandi F. Treatment of rare factor deficiencies other than hemophilia. Blood. 2019;133(5):415–424. doi: 10.1182/blood-2018-06-820738. - DOI - PubMed