Case Report
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Apr 6, 2021; 9(10): 2409-2418
Published online Apr 6, 2021. doi: 10.12998/wjcc.v9.i10.2409
Acquired haemophilia in patients with malignant disease: A case report
Veronika Krašek, Aleša Kotnik, Hana Zavrtanik, Jasna Klen, Samo Zver
Veronika Krašek, Aleša Kotnik, Hana Zavrtanik, Jasna Klen, Samo Zver, Medical Faculty, University of Ljubljana, Ljubljana 1000, Slovenia
Hana Zavrtanik, Jasna Klen, Department of Abdominal Surgery, University Medical Centre Ljubljana, Ljubljana 1000, Slovenia
Samo Zver, Department of Hematology, University Medical Centre Ljubljana, Ljubljana 1000, Slovenia
Author contributions: Klen J has conceived and designed the manuscript; Krašek V, Kotnik A and Zavrtanik H contributed to the acquisition, analysis and interpretation of the data; all authors participated in drafting the manuscript; Klen J and Zver S critically revised the manuscript; All authors read and approved the final version of the manuscript.
Informed consent statement: Written informed consent was obtained from the patient before the surgery.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest with regard to this case report.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Hana Zavrtanik, MD, Doctor, Department of Abdominal Surgery, University Medical Centre Ljubljana, Zaloška cesta 7, Ljubljana 1000, Slovenia. hana.zavrtanik@kclj.si
Received: December 21, 2020
Peer-review started: December 21, 2020
First decision: December 30, 2020
Revised: January 4, 2021
Accepted: January 26, 2021
Article in press: January 26, 2021
Published online: April 6, 2021
Processing time: 98 Days and 11.6 Hours
Abstract
BACKGROUND

Acquired haemophilia is a rare coagulation disorder characterized by autoantibodies against coagulation factor VIII leading to severe and potentially life-threatening haemorrhages. The underlying disorder causing the development of an autoimmune phenomenon is not always known, but 10%-15% could be linked to malignancies. Patients with cancer who require surgical resection represent a treatment challenge not solely due to increased risk of bleeding but also due to adverse events of immunosuppressive therapy.

CASE SUMMARY

We present the case of a 67-year-old man with non-metastatic adenocarcinoma of the distal bile duct who developed concomitant acquired haemophilia a month after having been diagnosed with malignant disease. Haemostasis was established with recombinant activated factor VII, and immunosuppressive therapy was started immediately. An extensive surgical procedure was performed in order to remove the cancer and, therefore, eliminate the inhibitory autoantibodies. Due to a complicated postoperative course, relatively short period of treatment and likelihood of micrometastases, no improvement in the patient’s status was observed. Diagnosis and treatment of acquired haemophilia as well as other coagulation disorders in patients with cancer are discussed.

CONCLUSION

Prompt diagnosis of acquired haemophilia is required in order to start appropriate treatment and reduce mortality. Among patients with cancer, other causes of abnormal bleeding related to malignancy should be considered.

Keywords: Malignancy; Haemostasis; Bleeding diathesis; Eradication therapy; Case report

Core Tip: Acquired haemophilia is a rare coagulation disorder characterized by autoantibodies against coagulation factor VIII. Immediate consultation with a reference haemophilia centre should be made for obtaining early diagnosis and treatment. In patients with cancer, a wide range of haemostatic disorders should be taken into consideration and distinguished from acquired haemophilia. Although invasive procedures should be avoided in patients with acquired haemophilia, surgical resection of the underlying malignancy is sometimes required. This subset of patients represents a treatment challenge not solely due to increased risk of bleeding but also due to adverse events of immunosuppressive therapy.