A case of acquired hemophilia A after pancreaticoduodenectomy for distal cholangiocarcinoma

Takahashi,Makoto; Morita,Yasuhiro; Hayashi,Tatsuya; Higashihara,Taku; Kawasaki,Keishi; Sato,Shunsuke; Yokose,Shota; Sasaki,Shu; Funakoshi,Kaoruko; Sasaki,Takayoshi; Zhou,Daren; Ichinose,Akinori; Ohtsuka,Hideo; Ishibashi,Yuji; Hatao,Fumihiko; Shimizu,Keiki; Isono,Nobuo; Sasaki,Naomi; Kozai,Yasuji; Okada,Haruka; Chikasawa,Yushi

doi:10.3892/br.2023.1643

A case of acquired hemophilia A after pancreaticoduodenectomy for distal cholangiocarcinoma

Authors:
- Makoto Takahashi
- Yasuhiro Morita
- Tatsuya Hayashi
- Taku Higashihara
- Keishi Kawasaki
- Shunsuke Sato
- Shota Yokose
- Shu Sasaki
- Kaoruko Funakoshi
- Takayoshi Sasaki
- Daren Zhou
- Akinori Ichinose
- Hideo Ohtsuka
- Yuji Ishibashi
- Fumihiko Hatao
- Keiki Shimizu
- Nobuo Isono
- Naomi Sasaki
- Yasuji Kozai
- Haruka Okada
- Yushi Chikasawa
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Affiliations: Department of Surgery, Tokyo Metropolitan Tama Medical Center, Tokyo 183‑8524, Japan, Department of Critical Care and Emergency Medicine, Tokyo Metropolitan Tama Medical Center, Tokyo 183‑8524, Japan, Department of Plastic Surgery, Tokyo Metropolitan Tama Medical Center, Tokyo 183‑8524, Japan, Department of Nursing, Tokyo Metropolitan Tama Medical Center, Tokyo 183‑8524, Japan, Department of Hematology, Tokyo Metropolitan Tama Medical Center, Tokyo 183‑8524, Japan, Department of Pathology, Tokyo Metropolitan Tama Medical Center, Tokyo 183‑8524, Japan, Department of Laboratory Medicine, Tokyo Medical University, Tokyo 183‑8524, Japan
Published online on: August 1, 2023 https://doi.org/10.3892/br.2023.1643
Article Number: 61
Copyright: © Takahashi et al. This is an open access article distributed under the terms of Creative Commons Attribution License.

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Abstract

Acquired hemophilia A (AHA) is a rare disease that results from factor VIII inhibitors causing abnormal coagulation, and certain cases may develop after highly invasive surgery. The present case study reports on a 68‑year‑old male patient who developed AHA after undergoing a subtotal stomach‑preserving pancreatoduodenectomy for distal cholangiocarcinoma. The patient experienced complications after surgery, requiring reoperation on postoperative day (PD) 5 due to rupture of the Braun's enterostomy. On PD 6, angiography was performed after bleeding was detected in the jejunal limb, but hemostasis occurred spontaneously during the examination. Bleeding was observed again on PD 8 and direct surgical ligation was performed. On PD 14, bleeding recurred in the jejunal limb and angiography was performed to embolize the periphery of the second jejunal artery. During the procedure, the prothrombin time was normal, but only the activated partial thromboplastin time was prolonged. A close examination of the coagulation system revealed a decrease in factor VIII levels and the presence of factor VIII inhibitors, resulting in the diagnosis of AHA. Administration of steroids was initiated on PD 15 and, in addition to daily blood transfusions, activated prothrombin complex concentrate was administered to achieve hemostasis. The patient was discharged from the intensive care unit on PD 36 but later developed an intractable labial fistula due to suture failure at the gastrojejunostomy site. As the use of factor VIII inhibitors continued despite the administration of steroids, cyclophosphamide (CPA) pulse therapy was added at PD 58. However, CPA was ineffective and the administration of rituximab was initiated on PD 98. After 12 courses of rituximab, the patient tested negative for factor VIII inhibitors on PD 219. On PD 289, labial fistula closure was performed with continuous replacement of factor VIII and the patient was discharged on PD 342.

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