Successful concurrent triple surgery in an adolescent patient with haemophilia A and inhibitors treated with recombinant factor VIIa

Janić, Dragana; Brdar, Radivoj; Krstic, Zoran; Jovanović, Nada; Dokmanović, Lidija; Rodić, Predrag; Petronić, Ivana; Antović, Jovan P.

doi:10.1111/j.1365-2516.2007.01485.x

Cited by 4 publications

(5 citation statements)

References 5 publications

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“…In addition, as these hemophilic patients age, not only hepatic malignancies but also bile duct cancers and pancreatic cancers are developing and requiring surgery if possible 11 . Although some case reports have been published in the abdominal surgical area, no systematic reports have been found in the updated literature 12‐15 …”

Section: Discussionmentioning

confidence: 99%

“…11 Although some case reports have been published in the abdominal surgical area, no systematic reports have been found in the updated literature. [12][13][14][15] The group of one of the authors reported the results of hepatectomy in hemophilia patients in 2018. 16 The study reported on 10 patients and showed no differences in operative time, blood loss, red blood cell transfusion rate, major complication rate, and mortality rate.…”

Section: Discussionmentioning

confidence: 99%

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National survey of hepatobiliary and pancreatic surgery in hemophilia patients in Japan

Yoshimoto-Haramura

Hidaka

Hasegawa

et al. 2021

J Hepato Biliary Pancreat

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Background Approximately 8300 hemophiliacs are registered in Japan, but no comprehensive reports on hepatobiliary and pancreatic surgery (HBPS) have been conducted. This report investigates the current status of HPBS in hemophilia patients in Japan. Methods The subjects were hemophiliac patients seen between January 1 2007, and December 31 2017, at facilities participating in this study among the facilities for performing high‐difficulty cases nationwide designated by the Japanese Society for HBPS. A retrospective examination of short‐term outcomes in 49 cases was conducted to assess patient background, disease, surgical procedure, and complications. Results The types of hemophilia were A: 43 cases, B: four cases, and von Willebrand disease: two cases (hemophilia severity: mild 32, moderate seven, severe 10). The target malignant diseases for surgery were hepatocellular carcinoma (HCC) in 20 cases, intrahepatic cholangiocellular carcinoma (CCC) in four cases, combined HCC‐CCC in two cases, hilar CCC in two cases, and pancreatic cancer in four cases. As for the surgical procedure, limited resection (subsegmentectomy and partial hepatectomy) was performed in 16 cases of HCC even with normal liver function tests. Pancreaticoduodenectomy and distal pacreatectomy were performed for pancreatic cancers as in the standard procedure. Postoperative complications were postoperative bleeding in two cases after hepatectomy and one after pancreatectomy in one case. When compared with Japanese National Clinical Data base, the complication rates after hepatectomy and pancreatectomy were not conspicuous in hemophilic patients. Conclusions As long as they are performed in qualified centers, complication rate is not increased in hemophilic patients undergoing HBPS.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

National survey of hepatobiliary and pancreatic surgery in hemophilia patients in Japan

Yoshimoto-Haramura

Hidaka

Hasegawa

et al. 2021

J Hepato Biliary Pancreat

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“…By cross‐checking relevant articles, additional articles were added to the list in a non‐standardized manner. Almost 60 articles were identified describing 332 EOS procedures [7,9–36,41,43–58,60–69,73–75]. Based on the classification of Perez Bianco et al.…”

Section: Discussionmentioning

confidence: 99%

Elective orthopaedic surgery for haemophilia patients with inhibitors: single centre experience of 40 procedures and review of the literature

et al. 2011

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With the introduction of safe and effective factor VIII/IX-bypassing agents--recombinant activated factor VII (rFVIIa) and plasma-derived activated prothrombin complex concentrates (pd-APCC)--elective orthopaedic surgery (EOS) is a viable option for haemophilia patients with inhibitors. We report a series of patients with haemophilia and inhibitors undergoing EOS between 1997 and 2008 using bypassing agents to provide haemostatic cover. All inhibitor patients undergoing EOS and receiving rFVIIa, plasma-derived prothrombin complex concentrates (pd-PCC) or pd-APCC as haemostatic cover were included. Patients were operated on by the same surgeon and were managed by the same haemophilia treatment centre. Forty procedures (25 minor and 15 major) were conducted in 18 patients. Twenty-one minor cases were covered using rFVIIa, three with pd-PCC, and one with pd-APCC; all major cases were covered using rFVIIa. Bleeding was no greater than expected compared with a non-haemophilic population in all 25 minor procedures. In the major procedure group, there was no excessive bleeding in 40% of cases (6/15) and bleeding completely stopped in response to rFVIIa. For the remaining nine cases, bleeding response to rFVIIa was described as 'markedly decreased' or 'decreased' in 4/15 cases and 'unchanged' in 5/15 cases. Overall, efficacy of rFVIIa, based on final patient outcome, was 85%. One death occurred as a result of sepsis secondary to necrotizing fasciitis. Good control of haemostasis can be achieved with bypassing agents in haemophilia patients with inhibitors undergoing minor EOS; rFVIIa was used as an effective bypassing agent, enabling EOS in patients undergoing minor and major procedures.

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“…Only English language literature was considered. Fifty articles were subsequently identified [10–57]. Underlying coagulopathy and/or the results of rFVIIa treatment in congenital haemophilia patients with inhibitors could be ascertained in only 46 of the 50 articles identified.…”

Section: Methodsmentioning

confidence: 99%

Surgical Experience with rFVIIa (NovoSeven) in congenital haemophilia A and B patients with inhibitors to factors VIII or IX

2011

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Patients with congenital haemophilia with inhibitors are at risk of peri-operative bleeding complications, since replacement of the missing coagulation factor is ineffective, presenting a therapeutic challenge in elective or emergency surgery. Therefore, the management of peri-operative bleeding requires the use of bypassing agents, such as recombinant activated FVII (rFVIIa, NovoSeven(®) ). This article presents an updated evaluation of the safety and effectiveness of rFVIIa in the treatment of peri-operative bleeding in this patient population. Surgical and other medical procedures managed with rFVIIa from two randomized clinical trials, the Hemophilia Research Society/Hemophilia and Thrombosis Research Society (HRS/HTRS) registry databases and the medical literature were analysed. There were 395 rFVIIa-treated procedures (261 surgical, 89 dental and 45 other medical procedures) reported for 263 congenital haemophilia patients with inhibitors. In trials, initial rFVIIa dosing was 35-90 mcg kg(-1) bolus injection or 50 mcg kg(-1) h(-1) continuous infusion. Dosing in the registries and literature was more variable. Recombinant FVIIa effectiveness was comparable across data sources, with an overall rate of 84% (333/395). The incidence of thrombotic events was very low (0.4% of patients and 0.025% of procedures). Prior to the US approval of rFVIIa in 1999, surgical procedures in congenital haemophilia patients with inhibitors were often considered too risky. Recombinant FVIIa has consistently demonstrated effectiveness in treatment of bleeding in these patients during such procedures. Thrombotic events were rare. This analysis confirms the value of corroborating clinical trial results with post-marketing surveillance registries to assess small patient populations with clinically challenging management decisions.

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Successful concurrent triple surgery in an adolescent patient with haemophilia A and inhibitors treated with recombinant factor VIIa

Cited by 4 publications

References 5 publications

National survey of hepatobiliary and pancreatic surgery in hemophilia patients in Japan

National survey of hepatobiliary and pancreatic surgery in hemophilia patients in Japan

Elective orthopaedic surgery for haemophilia patients with inhibitors: single centre experience of 40 procedures and review of the literature

Surgical Experience with rFVIIa (NovoSeven) in congenital haemophilia A and B patients with inhibitors to factors VIII or IX

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