[Multidisciplinary Management and Diagnostic Insights for Refractory Postoperative Hemorrhage in Mild Hemophilia B Caused by Hemizygous F9 c.881G>A (p.Arg294Gln) Variant].

OBJECTIVE

To explore perioperative management strategies for mild hereditary hemorrhagic disorders and analyze multidisciplinary collaborative treatment protocols for refractory postoperative bleeding caused by F9 gene variants.

METHODS

We retrospectively reviewed the management of a 31-year-old male with persistent hemorrhage after mixed hemorrhoidectomy. Diagnostic data including coagulation tests ［activated partial thromboplastin time (APTT), coagulation factor activity］, genetic sequencing, and transfusion records was analyzed to summarize key lessons.

RESULTS

The preoperative APTT was prolonged to 45.5 s (reference range: 22-38 s). Postoperative bleeding led to progressive hemoglobin decline (164→48 g/L), ultimately controlled with 10 U cryoprecipitate, 3 650 ml fresh frozen plasma (FFP), and 7.5 U red blood cells (RBC). Laboratory confirmation showed that factor IX (FIX) activity was 8.10%-10.10% (reference range: 60%-150%); un-corrected APTT mixing study; hemizygous pathogenic F9 c.881G>A (p.Arg294Gln) variant. Hemoglobin normalized to 121 g/L at 2-month follow-up, and no recurrence bleeding occurred.

CONCLUSION

APTT prolongation serves as a critical warning sign for mild hemophilia B, mandating preoperative coagulation factor screening in males. The p.Arg294Gln substitution disrupts FⅨ catalytic activity, triggering refractory surgical hemorrhage. Multidisciplinary collaboration (replacement therapy+anemia correction) successfully managed life-threatening bleeding, early genetic diagnosis could prevent the risk of postoperative bleeding.