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 & PLUSMN; 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 & PLUSMN; 1.3 months. The survival in combined FV and FVIII deficiencies was found to be longer than in the other deficiencies (55.9 & PLUSMN; 5.7), but there was no significant difference (P & GE; 0.05). In contrast, the survival in FXIII deficiency was observed to be lower than the other cases (44 & PLUSMN; 9.6); however, no significant difference was found in this regard (P & GE; 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.

Automated summary

This study aimed to assess the survival of patients with rare bleeding disorders (RBDs) in a five-year follow-up. The results showed that patients with RBDs had different rates of survival, and timely therapeutic interventions may improve their survival time.