Octocog-α

Octocog-α Reactions 1680, p262 - 2 Dec 2017 Factor rFVIII inhibitor development and rectal bleeding: case report An 81-year-old man developed factor rFVIII inhibitor and rectal bleeding during treatment with octocog-α [route not stated]. The man, who had mild FVIII deficiency, had his first exposure to the blood products at the age of 57 years in 1992. At that time, he had 22 exposure days (EDs) of intensive treatment with 93000 units of plasma derived factor VIII, for subdural haematoma following head trauma. He showed a good FVIII recovery. Three months following the therapy, inhibitor screen was negative. Three years later, at the age of 60 years, he was treated with octocog-α 44 U/kg daily for three days, for an elective skin lesion removal. Six years later, at the age of 66 years, he underwent hernia repair and received octocog-α 44 U/kg twice daily for approximately a week. Inhibitor screen tests were performed on an annual basis, which showed negative results. His next exposure was after seven years in 2008, at the age of 73 years. At that time he received octocog-α 44 U/kg prior and octocog-α 22 U/kg daily for nearly a week, after an extensive Mohs procedure. One week following the therapy completion, he received octocog-α 22 U/kg before and 12 hours after colonoscopy with polypectomy without immediate complications. Approximately six days following the polypectomy, he developed severe rectal bleeding, which was unresponsive to octocog-α 22 U/kg which was administered for five days. Due to the continued bleeding, he was treated with octocog-α 50 U/kg once daily for two days with suboptimal peak recovery levels of only 21%. At this point, he was exposed to octocog-α daily for 14 days over a span of three weeks for both Mohs procedure and colonoscopy (total 49 EDs). Following dose increase of octocog-α to 88 U/kg twice daily, the peak factor recovery was only 50–60%. At this time, after a total of 55 EDs over a span of 24 years, he developed a low titre inhibitor of 1.6 BU. The inhibitor resolved spontaneously in <1 months and did not reappeared despite repeated exposures in the next eight years. The repeated exposure included a single dose of octocog-α 22 U/kg for an endoscopy in 2013 in which his FVIII level increased appropriately to 65% with a negative Bethesda titre after a month. In 2016, similar clinical response was observed, when he received octocog-α 44 U/kg prior to and 22 U/kg daily for three days following excision of another skin lesion. NextGen sequencing of his F8 gene was performed, which showed a missense mutation (c.6113A>G) in exon 19 at codon 2019. The genetic predisposition to inhibitor development is closely linked to this underlying mutation. Author comment: "This case highlights the transient development of a low titre inhibitor with a severe bleeding phenotype following [octocog-α] given over 14 days for two minor surgical procedures in a man with mild Haemophilia A with 49 previous EDs over a period of 16 years." Evans MS, et al. Development of an inhibitor in a man with mild haemophilia A. Haemophilia 23: e473-e474, No. 5, Sep 2017. Available from: URL: http:// doi.org/10.1111/hae.13303 - USA 803284722 0114-9954/17/1680-0001/$14.95 Adis © 2017 Springer International Publishing AG. All rights reserved Reactions 2 Dec 2017 No. 1680 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Reactions Weekly Springer Journals

Octocog-α

Reactions Weekly , Volume 1680 (1) – Dec 2, 2017
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Publisher
Springer Journals
Copyright
Copyright © 2017 by Springer International Publishing AG, part of Springer Nature
Subject
Medicine & Public Health; Drug Safety and Pharmacovigilance; Pharmacology/Toxicology
ISSN
0114-9954
eISSN
1179-2051
D.O.I.
10.1007/s40278-017-39193-0
Publisher site
See Article on Publisher Site

Abstract

Reactions 1680, p262 - 2 Dec 2017 Factor rFVIII inhibitor development and rectal bleeding: case report An 81-year-old man developed factor rFVIII inhibitor and rectal bleeding during treatment with octocog-α [route not stated]. The man, who had mild FVIII deficiency, had his first exposure to the blood products at the age of 57 years in 1992. At that time, he had 22 exposure days (EDs) of intensive treatment with 93000 units of plasma derived factor VIII, for subdural haematoma following head trauma. He showed a good FVIII recovery. Three months following the therapy, inhibitor screen was negative. Three years later, at the age of 60 years, he was treated with octocog-α 44 U/kg daily for three days, for an elective skin lesion removal. Six years later, at the age of 66 years, he underwent hernia repair and received octocog-α 44 U/kg twice daily for approximately a week. Inhibitor screen tests were performed on an annual basis, which showed negative results. His next exposure was after seven years in 2008, at the age of 73 years. At that time he received octocog-α 44 U/kg prior and octocog-α 22 U/kg daily for nearly a week, after an extensive Mohs procedure. One week following the therapy completion, he received octocog-α 22 U/kg before and 12 hours after colonoscopy with polypectomy without immediate complications. Approximately six days following the polypectomy, he developed severe rectal bleeding, which was unresponsive to octocog-α 22 U/kg which was administered for five days. Due to the continued bleeding, he was treated with octocog-α 50 U/kg once daily for two days with suboptimal peak recovery levels of only 21%. At this point, he was exposed to octocog-α daily for 14 days over a span of three weeks for both Mohs procedure and colonoscopy (total 49 EDs). Following dose increase of octocog-α to 88 U/kg twice daily, the peak factor recovery was only 50–60%. At this time, after a total of 55 EDs over a span of 24 years, he developed a low titre inhibitor of 1.6 BU. The inhibitor resolved spontaneously in <1 months and did not reappeared despite repeated exposures in the next eight years. The repeated exposure included a single dose of octocog-α 22 U/kg for an endoscopy in 2013 in which his FVIII level increased appropriately to 65% with a negative Bethesda titre after a month. In 2016, similar clinical response was observed, when he received octocog-α 44 U/kg prior to and 22 U/kg daily for three days following excision of another skin lesion. NextGen sequencing of his F8 gene was performed, which showed a missense mutation (c.6113A>G) in exon 19 at codon 2019. The genetic predisposition to inhibitor development is closely linked to this underlying mutation. Author comment: "This case highlights the transient development of a low titre inhibitor with a severe bleeding phenotype following [octocog-α] given over 14 days for two minor surgical procedures in a man with mild Haemophilia A with 49 previous EDs over a period of 16 years." Evans MS, et al. Development of an inhibitor in a man with mild haemophilia A. Haemophilia 23: e473-e474, No. 5, Sep 2017. Available from: URL: http:// doi.org/10.1111/hae.13303 - USA 803284722 0114-9954/17/1680-0001/$14.95 Adis © 2017 Springer International Publishing AG. All rights reserved Reactions 2 Dec 2017 No. 1680

Journal

Reactions WeeklySpringer Journals

Published: Dec 2, 2017

References

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