Manifestations of factor VIII inhibitor in the head and neck

Frank J. DellaCroce, Stilianos E Kountakis, E. Fred Aguilar

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

Hemophilia A is the most severe of the inherited bleeding disorders. Otolaryngologists are frequently asked to assist in the care of a patient with this disorder who has intractable bleeding from sites in the head and neck. This disorder is transmitted in an X-linked recessive manner and results from factor VIII deficiency. Factor replacement therapy has contributed much to the management of acute bleeding episodes and the prevention of long-term sequelae in patients with hemophilia. A consequence of factor replacement therapy that occurs in as much as 16% of patients is the development of antibodies to the exogenous factor. These antibodies inactivate any supplemental factor VIII, resulting in continued bleeding despite attempts at replacement therapy. This poses an extremely challenging clinical problem because other standard therapies have had limited usefulness. Activated prothrombin complex has been shown to be efficacious in the treatment of patients with inhibitors. This has led to the production of factor VIII inhibitor bypassing activity (FEIBA), which contains activated forms of factors II, VII, IX, and X. The mechanism of action of this preparation remains unknown, but it seems to bypass the need for factor VIII in the clotting cascade. Factor VIII inhibitor bypassing activity is not as effective as factor VIII concentrates in patients with normally responsive hemophilia, and patients with low inhibitor levels may be given larger doses of factor VIII. Disseminated intravascular coagulation is most often mentioned as a potential complication of the administration of factor VIII inhibitor bypassing activity, although it has been rarely reported. Otolaryngologists should be familiar with this clinical phenomenon and understand its implications. Two patients with bleeding diatheses were recently treated at our institution.

Original languageEnglish (US)
Pages (from-to)1258-1261
Number of pages4
JournalArchives of Otolaryngology - Head and Neck Surgery
Volume125
Issue number11
DOIs
StatePublished - Jan 1 1999
Externally publishedYes

Fingerprint

Factor VIII
Neck
Head
Hemophilia A
Hemorrhage
Prothrombin
Therapeutics
Factor VII
Antibodies
Disseminated Intravascular Coagulation
Disease Susceptibility
Patient Care

ASJC Scopus subject areas

  • Surgery
  • Otorhinolaryngology

Cite this

Manifestations of factor VIII inhibitor in the head and neck. / DellaCroce, Frank J.; Kountakis, Stilianos E; Aguilar, E. Fred.

In: Archives of Otolaryngology - Head and Neck Surgery, Vol. 125, No. 11, 01.01.1999, p. 1258-1261.

Research output: Contribution to journalArticle

DellaCroce, Frank J. ; Kountakis, Stilianos E ; Aguilar, E. Fred. / Manifestations of factor VIII inhibitor in the head and neck. In: Archives of Otolaryngology - Head and Neck Surgery. 1999 ; Vol. 125, No. 11. pp. 1258-1261.
@article{f17f9cc24911436e954964f02d3a3795,
title = "Manifestations of factor VIII inhibitor in the head and neck",
abstract = "Hemophilia A is the most severe of the inherited bleeding disorders. Otolaryngologists are frequently asked to assist in the care of a patient with this disorder who has intractable bleeding from sites in the head and neck. This disorder is transmitted in an X-linked recessive manner and results from factor VIII deficiency. Factor replacement therapy has contributed much to the management of acute bleeding episodes and the prevention of long-term sequelae in patients with hemophilia. A consequence of factor replacement therapy that occurs in as much as 16{\%} of patients is the development of antibodies to the exogenous factor. These antibodies inactivate any supplemental factor VIII, resulting in continued bleeding despite attempts at replacement therapy. This poses an extremely challenging clinical problem because other standard therapies have had limited usefulness. Activated prothrombin complex has been shown to be efficacious in the treatment of patients with inhibitors. This has led to the production of factor VIII inhibitor bypassing activity (FEIBA), which contains activated forms of factors II, VII, IX, and X. The mechanism of action of this preparation remains unknown, but it seems to bypass the need for factor VIII in the clotting cascade. Factor VIII inhibitor bypassing activity is not as effective as factor VIII concentrates in patients with normally responsive hemophilia, and patients with low inhibitor levels may be given larger doses of factor VIII. Disseminated intravascular coagulation is most often mentioned as a potential complication of the administration of factor VIII inhibitor bypassing activity, although it has been rarely reported. Otolaryngologists should be familiar with this clinical phenomenon and understand its implications. Two patients with bleeding diatheses were recently treated at our institution.",
author = "DellaCroce, {Frank J.} and Kountakis, {Stilianos E} and Aguilar, {E. Fred}",
year = "1999",
month = "1",
day = "1",
doi = "10.1001/archotol.125.11.1258",
language = "English (US)",
volume = "125",
pages = "1258--1261",
journal = "JAMA Otolaryngology - Head and Neck Surgery",
issn = "2168-6181",
publisher = "American Medical Association",
number = "11",

}

TY - JOUR

T1 - Manifestations of factor VIII inhibitor in the head and neck

AU - DellaCroce, Frank J.

AU - Kountakis, Stilianos E

AU - Aguilar, E. Fred

PY - 1999/1/1

Y1 - 1999/1/1

N2 - Hemophilia A is the most severe of the inherited bleeding disorders. Otolaryngologists are frequently asked to assist in the care of a patient with this disorder who has intractable bleeding from sites in the head and neck. This disorder is transmitted in an X-linked recessive manner and results from factor VIII deficiency. Factor replacement therapy has contributed much to the management of acute bleeding episodes and the prevention of long-term sequelae in patients with hemophilia. A consequence of factor replacement therapy that occurs in as much as 16% of patients is the development of antibodies to the exogenous factor. These antibodies inactivate any supplemental factor VIII, resulting in continued bleeding despite attempts at replacement therapy. This poses an extremely challenging clinical problem because other standard therapies have had limited usefulness. Activated prothrombin complex has been shown to be efficacious in the treatment of patients with inhibitors. This has led to the production of factor VIII inhibitor bypassing activity (FEIBA), which contains activated forms of factors II, VII, IX, and X. The mechanism of action of this preparation remains unknown, but it seems to bypass the need for factor VIII in the clotting cascade. Factor VIII inhibitor bypassing activity is not as effective as factor VIII concentrates in patients with normally responsive hemophilia, and patients with low inhibitor levels may be given larger doses of factor VIII. Disseminated intravascular coagulation is most often mentioned as a potential complication of the administration of factor VIII inhibitor bypassing activity, although it has been rarely reported. Otolaryngologists should be familiar with this clinical phenomenon and understand its implications. Two patients with bleeding diatheses were recently treated at our institution.

AB - Hemophilia A is the most severe of the inherited bleeding disorders. Otolaryngologists are frequently asked to assist in the care of a patient with this disorder who has intractable bleeding from sites in the head and neck. This disorder is transmitted in an X-linked recessive manner and results from factor VIII deficiency. Factor replacement therapy has contributed much to the management of acute bleeding episodes and the prevention of long-term sequelae in patients with hemophilia. A consequence of factor replacement therapy that occurs in as much as 16% of patients is the development of antibodies to the exogenous factor. These antibodies inactivate any supplemental factor VIII, resulting in continued bleeding despite attempts at replacement therapy. This poses an extremely challenging clinical problem because other standard therapies have had limited usefulness. Activated prothrombin complex has been shown to be efficacious in the treatment of patients with inhibitors. This has led to the production of factor VIII inhibitor bypassing activity (FEIBA), which contains activated forms of factors II, VII, IX, and X. The mechanism of action of this preparation remains unknown, but it seems to bypass the need for factor VIII in the clotting cascade. Factor VIII inhibitor bypassing activity is not as effective as factor VIII concentrates in patients with normally responsive hemophilia, and patients with low inhibitor levels may be given larger doses of factor VIII. Disseminated intravascular coagulation is most often mentioned as a potential complication of the administration of factor VIII inhibitor bypassing activity, although it has been rarely reported. Otolaryngologists should be familiar with this clinical phenomenon and understand its implications. Two patients with bleeding diatheses were recently treated at our institution.

UR - http://www.scopus.com/inward/record.url?scp=0032697878&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0032697878&partnerID=8YFLogxK

U2 - 10.1001/archotol.125.11.1258

DO - 10.1001/archotol.125.11.1258

M3 - Article

C2 - 10555699

AN - SCOPUS:0032697878

VL - 125

SP - 1258

EP - 1261

JO - JAMA Otolaryngology - Head and Neck Surgery

JF - JAMA Otolaryngology - Head and Neck Surgery

SN - 2168-6181

IS - 11

ER -