Lab Med Online.  2021 Jul;11(3):203-207. 10.47429/lmo.2021.11.3.203.

Effective Management of a Patient with Acquired Coagulation Factor Inhibitors Using Therapeutic Plasma Exchange Alone

• Na R
• Ryu KS
• Kim MS
• Yang JJ
• Park HS
• Jang S
• Hwang SH
• Oh HB
• Ko DH

• KMID: 2526071
• DOI: http://doi.org/10.47429/lmo.2021.11.3.203

Abstract

Acquired coagulation factor inhibitors are autoantibodies that mainly inhibit the action of coagulation factors, and prompt diagnosis and treatment are important in this situation. Treatment often consists of suppression of hemorrhage, elimination of autoantibodies, and correction of the underlying cause. Therapeutic plasma exchange (TPE) is used as an adjuvant treatment in patients with acquired coagulation factor inhibitors; however, there is no consensus about its clinical significance. Herein, we reported a case of a 70-year-old female patient with acquired coagulation factor inhibitors treated with 13 sessions of TPE after antibiotic treatment. The presence of inhibitors was confirmed by the increased coagulation times without correction in the mixing tests and a factor VIII inhibitor level of 12 Bethesda U/mL. Since the patient could not undergo conventional immunosuppressive therapy because of suspected infective endocarditis and spinal osteomyelitis, only TPE was performed with a successful outcome. To our knowledge, this is the first case where acquired coagulation factor inhibitors were managed by TPE alone. Therefore, TPE might be an effective treatment option in patients with acquired coagulation factor inhibitors when conventional immunosuppressive therapy cannot be applied.

Keyword

Acquired coagulation factor inhibitor; Therapeutic plasma exchange; Antibiotic

Figure

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  Fig. 1 Changes in prothrombin time (PT), activated partial thromboplastin time (aPTT) after therapeutic plasma exchange. Dashed and solid lines indicate PT (INR) and aPTT, respectively. Arrows indicate the days on which therapeutic plasma exchange was performed. The antibiotics used included ampicillin (AMP)+ceftriaxone (CRO) from hospitalization day (HD) 1 to HD 20 and vancomycin (VAN) from HD 21 until patient discharge.

Reference

1. Padmanabhan A, Connelly-Smith L, Aqui N, Balogun RA, Klingel R, Meyer E, et al. 2019; Guidelines on the use of therapeutic apheresis in clinical practice - evidence-based approach from the writing committee of the American Society for Apheresis: the eighth special issue. J Clin Apher. 34:171–354. DOI: 10.1002/jca.21705. PMID: 31180581.

2. Franchini M, Capra F, Nicolini N, Veneri D, Manzato F, Baudo F, et al. 2007; Drug-induced anti-factor VIII antibodies: A systematic review. Med Sci Monit. 13:RA55–61. DOI: 10.1002/chin.200745229.

3. Borg JY, Guillet B, Le Cam-Duchez V, Goudemand J, Lévesque H. Group SS. 2013; Outcome of acquired haemophilia in France: the prospective SACHA (Surveillance des Auto antiCorps au cours de l'Hémophilie Acquise) registry. Haemophilia. 19:564–70. DOI: 10.1111/hae.12138. PMID: 23574453.

4. Franchini M, Vaglio S, Marano G, Mengoli C, Gentili S, Pupella S, et al. 2017; Acquired hemophilia A: a review of recent data and new therapeutic options. Hematology. 22:514–20. DOI: 10.1080/10245332.2017.1319115. PMID: 28441921.

5. Lim DH, Kim TO, Jeong Y, Kim WJ, Park SJ, Lee JH, et al. 2014; Antibiotic-induced acquired factor V Inhibitor. Korean J Med. 87:105–9. DOI: 10.3904/kjm.2014.87.1.105.

6. Wool GD, Chapel D, Treml A, Miller JL. 2017; Therapeutic plasma exchange as part of multimodal treatment of acquired hemophilia in a patient with concurrent acute intracerebral bleed and pulmonary embolism. Transfusion. 57:1827–32. DOI: 10.1111/trf.14132. PMID: 28436106.

7. Kumari S, Tripathi PP, Sharma RR, Hans R, Lamba DS, Khadwal A, et al. 2020; Efficacy of therapeutic plasma exchange in a patient with coagulation inhibitors (acquired haemophilia A) - A case report. Transfus Apher Sci. 59:102809. DOI: 10.1016/j.transci.2020.102809. PMID: 32527615.

8. Kim YK, Suh JS. 2012; Acquired hemophilia: successfully treated with plasma exchange in a patient with hightiter inhibitors. Korean J Blood Transfus. 23:68–71.