Is major surgery in hemophiliac patients safe?

Rudowski, W; R, Scharf; Ziemski, J.M.

doi:10.1007/bf01658122

Cited by 29 publications

(9 citation statements)

References 34 publications

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“…In haemophiliac patients, the reported postoperative bleeding complication rate is 3%–6%, and the postoperative infection rate about 1%,4 confirmed also in this study.…”

Section: Discussionsupporting

confidence: 89%

General surgery in haemophiliac patients

Bastounis

Pikoulis²,

Leppäniemi³

et al. 2000

Postgraduate Medical Journal

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With improvements in medical technology, more and larger surgical procedures are performed in haemophiliac patients, but rarely reported in the surgical literature. A retrospective study from a 10 year period from one referral centre identified a total of 68 operations performed in haemophiliac patients. The levels of the defective factors were carefully monitored preoperatively and postoperatively, and replaced according to a standard formula. Special caution was taken to avoid any postoperative medication or procedures that could provoke haemorrhagic complications.Two patients suVered postoperative bleeding complications that were managed conservatively. Inhibitory factors were detected preoperatively in one case, and postoperatively in another, and were managed with aggressive replacement therapy. There was no mortality, and the overall morbidity rate was 6%.With adequate preoperative and postoperative monitoring of the clotting factors, meticulous haemostasis during surgery, careful postoperative nursing, and timely replacement therapy, haemophiliac patients can be operated with good results. Postoperative bleeding complications are rare, and usually amenable to conservative management. (Postgrad Med J 2000;76:494-495)

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“…In haemophiliac patients, the reported postoperative bleeding complication rate is 3%–6%, and the postoperative infection rate about 1%,4 confirmed also in this study.…”

Section: Discussionsupporting

confidence: 89%

General surgery in haemophiliac patients

Bastounis

Pikoulis²,

Leppäniemi³

et al. 2000

Postgraduate Medical Journal

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“…Overall, the amount of rFIXFc used to maintain haemostasis during the perioperative period was less than that observed in historical studies of conventional FIX products (Rudowski et al , ; Ragni et al , ; Quon & Logan, ). In B‐LONG, the mean dose on the day of surgery (Day 0) was 84·16 iu/kg; mean doses on subsequent days (Days 1–14) ranged from 49·12–64·61 iu/kg.…”

Section: Discussionmentioning

confidence: 72%

Long‐acting recombinant factor IX Fc fusion protein (rFIXFc) for perioperative management of subjects with haemophilia B in the phase 3 B‐LONG study

et al. 2014

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SummaryIn the phase 3 B-LONG (Recombinant Factor IX Fc Fusion Protein [rFIXFc] in Subjects With Haemophilia B) study, rFIXFc demonstrated a prolonged half-life compared with recombinant factor IX (rFIX), and safety and efficacy for prophylaxis and treatment of bleeding in subjects with moderately-severe to severe haemophilia B. In this B-LONG sub-analysis, rFIXFc was evaluated for efficacy in subjects requiring major surgery. Dosing was investigator-determined. Assessments included dosing, consumption, bleeding, transfusions and haemostatic response. A population pharmacokinetics model of rFIXFc was used to predict FIX activity. Twelve subjects underwent 14 major surgeries (including 11 orthopaedic surgeries); most subjects (11/12) received rFIXFc prophylaxis before surgery (range, 2 weeks-12 months). Investigators/surgeons rated haemostatic responses as excellent (n = 13) or good (n = 1). In most surgeries (85Á7%), haemostasis from the pre-surgical dose until the end of surgery was maintained with a single rFIXFc infusion. Blood loss was consistent with similar surgeries in subjects without haemophilia. The strong correlation (R 2 = 0Á9586, P < 0Á001) between observed and population pharmacokinetic model-predicted FIX activity suggests surgery did not impact rFIXFc pharmacokinetics. No unique safety concerns or inhibitors were observed. In conclusion, rFIXFc was safe and efficacious, with prolonged dosing intervals and low consumption, when used perioperatively in haemophilia B. Surgery did not appear to alter rFIXFc pharmacokinetics.

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“…4 In the current case, the abnormal bleeding was finally controlled using recombinant factor VIII preparations developed in 1993, in which the risk of a viral infection due to human immunodeficiency virus or hepatitis viruses has been minimized. 5 Regarding the dose of factor VIII preparations to be used in severe bleeding, we recommend that a loading dose of 50 U/kg factor VIII followed by 25 U/kg every 12 h should generally be sufficient.…”

Section: Discussionmentioning

confidence: 96%

“…1 As a result, this disease is relatively rare, and there have been few reports on thoracic surgery in patients with hemophilia A. [2][3][4] Three categories of severity in hemophilia A may be distinguished as follows: (1) severe deficiency (factor VIII activity of 0%-1%), (2) moderate deficiency (factor VIII activity of 2%-4%), and (3) mild deficiency (factor VIII activity of 5%-50%). The factor VIII activity of our patient was 23%, and as such was classified as a mild deficiency type of hemophilia A.…”

Section: Discussionmentioning

confidence: 99%

Bilateral Pneumothorax Accompanied by Mild Deficiency Type Hemophilia A: Report of a Case

Kitami

Suzuki

et al. 2003

Surgery Today

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Hemophilia A is relatively rare and the number of patients with this disorder who undergo surgery is limited. We herein report a surgical case of bilateral pneumothorax accompanied by mild deficiency type of hemophilia A. Although this patient had undergone major surgery at another institution, a diagnosis of hemophilia A had not been made, nor had he noticed unusual bleeding in his daily life. Laboratory studies showed a slight prolongation of the activated partial thromboplastin time. A thoracoscopic operation was performed for bilateral pneumothorax in 1996. After the initial operation, the blood discharge from the right thoracic drainage tube continued. Despite two reoperations, oozing from the chest wall could not be controlled. Finally, the patient had achieved a stable condition with hemothorax encompassing the right thorax. After a diagnosis of hemophilia A was made, we removed any coagulated blood while administering a factor VIII preparation. Clinical and laboratory indications in daily life of mild deficiency type hemophilia A are not manifest in comparison with those of severe or moderate types. It is therefore necessary to consider this disorder in cases of abnormal bleeding at or after operation.

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Is major surgery in hemophiliac patients safe?

Cited by 29 publications

References 34 publications

General surgery in haemophiliac patients

General surgery in haemophiliac patients

Long‐acting recombinant factor IX Fc fusion protein (rFIXFc) for perioperative management of subjects with haemophilia B in the phase 3 B‐LONG study

Bilateral Pneumothorax Accompanied by Mild Deficiency Type Hemophilia A: Report of a Case

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