Case Report
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Nov 6, 2021; 9(31): 9592-9597
Published online Nov 6, 2021. doi: 10.12998/wjcc.v9.i31.9592
Forearm compartment syndrome due to acquired hemophilia that required massive blood transfusions after fasciotomy: A case report
Takuya Kameda, Takeru Yokota, Soichi Ejiri, Shin-ichi Konno
Takuya Kameda, Takeru Yokota, Shin-ichi Konno, Department of Orthopaedic Surgery, Fukushima Medical University, Fukushima 960-1295, Fukushima, Japan
Soichi Ejiri, Department of Hand and Limb Reconstructive Surgery, Fukushima Medical University, Fukushima 960-1295, Fukushima, Japan
Author contributions: Kameda T collected clinical data and wrote the paper; Kameda T, Yokota T and Ejiri S managed the patient; Ejiri S and Konno S critically reviewed the manuscript; all authors approved the final version.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: All authors do not have any conflict of interest.
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: Takuya Kameda, MD, PhD, Assistant Professor, Department of Orthopaedic Surgery, Fukushima Medical University, 1 Hikarigaoka, Fukushima 960-1295, Fukushima, Japan. a0001826@fmu.ac.jp
Received: April 24, 2021
Peer-review started: April 24, 2021
First decision: May 24, 2021
Revised: June 20, 2021
Accepted: October 11, 2021
Article in press: October 11, 2021
Published online: November 6, 2021
Processing time: 187 Days and 22.8 Hours
Abstract
BACKGROUND

Acquired hemophilia is rare. In some cases, the bleeding in muscle causes compartment syndrome. However, it is not clear whether fasciotomy should be performed for the compartment syndrome caused by acquired hemophilia because of the risk of bleeding and the unknown functional results.

CASE SUMMARY

A 75-year-old woman was admitted with severe pain of the right forearm with no preceding traumatic event. The right forearm was obviously swollen, and stretch pain was observed. Subcutaneous hematomas were suspected in various parts of the body. Compartment pressure was 110 mmHg on the volar side. Activated partial thromboplastin time (aPTT) was prolonged to 54.9 s. Fasciotomy was performed, and hematoma was observed in the volar compartment. Postoperative laboratory examinations revealed a low level of factor VIII (FVIII) activity (12.5%) and a high level of FVIII inhibitor (15.2 bethesda units/mL). Acquired hemophilia A was diagnosed. Though recombinant clotting factors were administered, transfusion of red blood cells reached 46 units (140 mL/unit). Hemostasis was achieved 9 d after fasciotomy. The total cost of the clotting factor concentrates administered reached 28834600 yen. With prednisolone, FVIII activity and aPTT recovered gradually. Final function of the hand was good in the index finger and excellent in the others.

CONCLUSION

Fasciotomy resulted in good function of the hand in a case of non-traumatic compartment syndrome caused by acquired hemophilia, but life-threatening bleeding occurred, and the cost of clotting factor treatment was high. Preparation of sufficient blood transfusion, preoperative administration of recombinant activated clotting factor VII, and prompt fasciotomy could be ideal for such cases.

Keywords: Acquired hemophilia; Compartment syndrome; Fasciotomy; Bleeding; Cost; Case report

Core Tip: Whether we should perform fasciotomy for compartment syndrome caused by acquired hemophilia is unclear. A 75-year-old woman admitted with severe pain of the right forearm was diagnosed as having compartment syndrome, and fasciotomy was performed. Laboratory data showed acquired hemophilia A. Though recombinant activated clotting factor VII (rFVIIa) was administered, transfused red blood cells reached 46 units, and the cost of the clotting factor was 28834600 yen. Final hand function was good, but life-threatening bleeding and the high cost of treatment were serious problems. Preparation for sufficient blood transfusion, preoperative administration of rFVIIa, and prompt fasciotomy could be ideal.