RESUMEN
Hemophilia is a recessive inherited disorder linked to the X chromosome. On July 28, 2020, a case of hemophilia A with severe left kidney hydronephroses and acute left kidney hemorrhage was admitted to the Affiliated Hospital of Qinghai University. The patient had a family history of hemophilia A and activated partial thromboplastin before surgery. The APTT was 95.0s (reference value 22.7-31.8s), factor Ⅷ activity (FⅧ∶C) was 3.2% (reference value 70%-150%), and B-ultrasonic examination revealed severe hydronephroses in the left kidney, and the nephrogram indicated that the left kidney was absent of perfusion. On the 9th day of admission, the pain in the left lower back was aggravated than before. The CT scan of the whole abdomen revealed irregular patches with slightly higher density in the left kidney. Considering bleeding, through multidisciplinary consultation and perioperative infusion of human coagulation factor Ⅷ, the patient was underwent retroperitoneal laparoscopic left nephrectomy. There was no bleeding during and after operation. After 20 months of follow-up, the renal function and coagulation function were normal, and there were no symptoms of hematuria and low back pain.