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

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.