Mild Hemophilia:2 Cases Report And Literature Review

OBJECTIVE:To improve the diagnosis and treated ability to mild hemophilia.METHODS:To discuss the medical history,clinical charactstics,dia gnosis,therapy of 2 cases patients with MHA,who were diagnosed and trea ted in The First Affiliated Hospital of Guangxi Medical University.RESULTS:2 cases of mild hemophilia patients are both males an d aged 18-19 years old.The first clinical symptom of the patients was mu scle hematoma after injury.When the results of their health examination re ported blood composition and coagulation function are normal,the patients undergone surgical interventions removing muscle hematoma.After the surg ery,the bleeding was no limited..One of the patients admitted to the intens ive care unit due to hemorrhagic shock.During the disease,the level of both patients' F? fluctuated between5% and 40%and APTT was normal or slightly increased.One of the patients detected mild hemophilia-relat ed gene mutation.After diagnosed,plasma-derived F?,recombinant acti vated factor VII and tranexamic acid were used to improve the function of coagulation,and plasma-derived F?,recombinant activated factor VIIwere used to prevent surgical bleeding.One patient developed inhibitor s during the disease,further,F? decreased and APTT was significantly prolonged.But there is no increase in bleeding observed.Both patients suc cessfully completed the surgical interventions and the wound recovered.CLUSIONS:1.Frequent spontaneous bleeding is rare in mild haemophilia.Patients may not bleed excessively unless they experience trauma or undergo a surgical intervention.2.Laboratory examination of PLT?PT and Figare normal and APTT is normal or slightly increased,which makes MHA invisible,therefore a factor assay and genetic testing can help for diagnosis.3.DDAVP is the first choice for mild hemophilia treatment,and F? concentrate is still the classic choice for treatment,which can effectively prevent major bleeding.4.Developing inhibitors may aggravate the bleeding phenotype,doctors should be cautious with the use of F? concentrate.