In a physical examination, the 60-year-old Mr. Ren was found to have a liver mass, which increased significantly during the follow-up consultation and was considered to be probably malignant. Mr. Ren had previously suffered from severe hemophilia with severe liver cirrhosis, and hemophilia was once an absolute contraindication for surgery as it made it very difficult to stop mass bleeding once it occurred during surgery. This is the first time that the Department of Liver Surgery of PUMCH has encountered such a case, and it is also very rare globally. The consultation for difficult hepatobiliary malignancies was launched quickly. With the support of the Department of Hematology, the Department of Transfusion, the operating room of the Department of Anesthesiology, and the Department of Intensive Care Unit, the Department of Liver Surgery successfully performed the left hepatic tumor resection for the patient. The patient recovered well after the operation and was discharged 12 days later.

At the age of 1, Mr. Ren was diagnosed with hemophilia A and needed intravenous infusion of Factor VIII every other day to improve blood coagulation. At the age of 58, his hemophilia-related arthritis symptoms worsened and he could only walk as far as about 100 meters. At the age of 59, his physical examination revealed a circular, slightly low-density shadow in the left lateral lobe of the liver, about 3.8cm×3.6cm in size, accompanied by abdominal distension. The re-examination found that the mass was significantly increased to 6.7cm×5.8cm. Mr. Ren was eager to receive treatment as soon as possible to relieve his pain.

The Department of Liver Surgery quickly organized a consultation for difficult hepatobiliary malignancies. Multidisciplinary specialists from the Department of Hematology, the Department of Oncology, the interventional therapy team under the Department of Radiology, the Department of Radiotherapy, and the Department of Liver Surgery gathered together and reached a consensus on the treatment: considering that the patient’s liver tumor occupies a large space and is combined with hemophilia and that his liver cirrhosis and portal hypertension are severe and complicated, the open left hepatic tumor resection is recommended, preceded by a preliminary test of coagulation factor replacement therapy.

Hemophilia is a genetic coagulation disorder, making patients susceptible to severe bleeding throughout their life, especially during the operation of organs with rich blood supply such as the liver, which makes intraoperative bleeding all the more difficult to stop once it occurs. For Mr. Ren, the risk of bleeding in the perioperative period is further increased due to the combination of liver cirrhosis, hypersplenism, and thrombocytopenia.

With the advancement of medical technology, academic reports of surgical operations on hemophilia patients have gradually increased; however, the cases of surgical operations on patients with hepatocellular carcinoma, liver cirrhosis and hemophilia at the same time are still very rare, and there is no mature experience to draw upon, let alone a guide to follow.

After making adequate preparations, the liver surgery team, with the cooperation of multidisciplinary specialists from such departments as Intensive Care Unit and Department of Anesthesiology and operating room nurses, completely removed the tumor within less than 4 hours, and the intraoperative blood loss was about 200ml. After the surgery, the patient was given replacement therapy that supplemented platelets and coagulation factor to ensure wound healing as well as nutritional boost, liver protection and other treatments. The patient recovered well and was discharged after 12 days. The pathological result is moderately differentiated hepatocellular carcinoma.

The success of this operation not only saved Mr. Ren’s life, but also was a cutting-edge exploration in the medical community, proving that under the multidisciplinary model, through adequate preoperative assessment and preparation, meticulous intraoperative operation and careful postoperative management, it is safe and feasible to operate on cirrhotic patients with hemophilia.

Multidisciplinary specialists were discussing the patient’s conditions.

The team led by associate chief physicians Zheng Yongchang and Xu Haifeng were operating on the patient.

Reporter: Gan Dingzhu

Correspondent: Yang Fan

Picture: The Department of Liver Surgery

Translator: Liu Haiyan

Editor: Xu Haifeng and Wang Yao