A Single Center Study On Perioperative Management Of Rare Coagulation Disorders Clinical Analysis Of 83 Cases Of Rare Coagulation Disorders

Objective: Rare coagulation disorders(RCDs)are autosomal recessive inherited diseases caused by one or more coagulation factor defects,including the deficiency of fibrinogen(FG),prothrombin,factor(F)V,VII,X,XI and XIII,as well as the combined deficiency of FV + FVIII and vitamin K-dependent coagulation factor(VKDCFD).The prevalence of RCDs in population is very low,ranging from 1/500000～1/3000000,and its clinical manifestations are very different.Due to the rarity and clinical heterogeneity of RCDs,there are lack of large-scale randomized controlled studies in the world,which limits the development of experimental diagnosis and treatment methods of this series of diseases.The situation makes it more difficult for perioperative management of RCDs,making it great challenge to achieve standardization of replacement treatment and monitoring evaluation.This study is a single-center retrospective study with a large number of samples in China.To some extent,it reflects the epidemiological characteristics,clinical characteristics,laboratory diagnosis and treatment status of RCDs in China.And we actively explore the risk factors of perioperative bleeding after replacement treatment,so as to better predict the risk of bleeding and standardize perioperative management.Methods: The clinical data,laboratory diagnosis,perioperative treatment and outcome of83 patients with RCDs were analyzed retrospectively.The corresponding statistical methods were used to describe the indicators objectively and analyze the risk factors of perioperative bleeding.Results: Among the 83 patients with RCDs,20 were male(24.1%)and 63 were female(75.9%);the age was 18～71 years(40.16 ± 14.20);55(66.3%)were severe deficiency,28(33.7%)were moderate deficiency and 0(0.0%)was mild deficiency.58 patients(58 / 83,69.9%)had no bleeding tendency in the past,while 25 patients(25 / 83,30.1%)suffered bleeding before admission,mainly including menorrhagia,trauma / invasive hemostasis difficulty and oral mucosal bleeding.83 patients were treated with replacement treatment,some patients were treated with antifibrinolytic drugs as complementary therapy.We observed that 77 patients(92.8%)did not suffer perioperative bleeding,and 6 patients(7.2%)experienced more intra-operative bleeding.Further logistic regression analysis showed that the bleeding history was an independent risk factor for intra-operative bleeding(P = 0.021,α = 0.05),and the relative risk value(odds ratio,OR)was 13.33.However,there was no significant difference between perioperative bleeding events after replacement treatment and degree of coagulation factor deficiency,the treatment,the preoperative evaluation and the use of antifibrinolytic drugs.Conclusion : This study shows that the prevalence of RCDs in the group and the composition of each coagulation factor deficiency are basically consistent with the data reported abroad.The clinical manifestations of RCDs are highly heterogeneous.It is difficult to judge the specific bleeding risk by the residual activity of coagulation factors in patients’ plasma.Our perioperative replacement treatment is effective in more than 90% of the cases,only 7.2% of them suffer more bleeding in operations,but no fatal bleeding occurred.And the bleeding history is the only independent predictor of intra-operative bleeding through statistical analysis.In addition,the impact of RCDs on women may be more serious.It is necessary to improve more understanding of gynecologists and surgeons on menorrhagia and perioperative hemostasis in women RCDs patients.