Flap cover in a patient with severe haemophilia type A

Haemophilia A is a rare haematological disorder due to deficiency of Factor VIII, causing an abnormal coagulation response to injury. In severe haemophilia A, Factor VIII level is <1%, often manifesting with spontaneous bleeding into joints. Judicious use of recombinant Factor VIII therapy to mai...

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Main Authors: Narender Manickavachakan, Sunderraj Ellur, Vijay Thomas Mattyoo Joseph, Jonathan Victor, Cecil R. Ross
Format: Article
Language:English
Published: Thieme Medical Publishers, Inc. 2017-05-01
Series:Indian Journal of Plastic Surgery
Subjects:
Online Access:http://www.thieme-connect.de/DOI/DOI?10.4103/ijps.IJPS_214_16
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author Narender Manickavachakan
Sunderraj Ellur
Vijay Thomas Mattyoo Joseph
Jonathan Victor
Cecil R. Ross
author_facet Narender Manickavachakan
Sunderraj Ellur
Vijay Thomas Mattyoo Joseph
Jonathan Victor
Cecil R. Ross
author_sort Narender Manickavachakan
collection DOAJ
description Haemophilia A is a rare haematological disorder due to deficiency of Factor VIII, causing an abnormal coagulation response to injury. In severe haemophilia A, Factor VIII level is <1%, often manifesting with spontaneous bleeding into joints. Judicious use of recombinant Factor VIII therapy to maintain adequate levels in the intraoperative, immediate and late post-operative periods, together with adjuvant pro-coagulants, can ensure a safe outcome following surgery. We describe the successful management of one such patient suffering from Marjolin's ulcer of the right gluteal region, who needed wide local excision followed by flap cover. A protocol for management of such patients is also suggested. This is the first such case report from the Indian subcontinent, with only a few such published reports from the West.
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spelling doaj.art-41d8f9bbd43b4f0bbae98a0aae35f06c2022-12-22T02:21:48ZengThieme Medical Publishers, Inc.Indian Journal of Plastic Surgery0970-03581998-376X2017-05-01500221321610.4103/ijps.IJPS_214_16Flap cover in a patient with severe haemophilia type ANarender Manickavachakan0Sunderraj Ellur1Vijay Thomas Mattyoo Joseph2Jonathan Victor3Cecil R. Ross4Department of Plastic Surgery and Hospital, Bengaluru, Karnataka, IndiaDepartment of Plastic Surgery and Hospital, Bengaluru, Karnataka, IndiaDepartment of Plastic Surgery and Hospital, Bengaluru, Karnataka, IndiaDepartment of Plastic Surgery and Hospital, Bengaluru, Karnataka, IndiaDepartment of Medicine, St. John's Medical College and Hospital, Bengaluru, Karnataka, IndiaHaemophilia A is a rare haematological disorder due to deficiency of Factor VIII, causing an abnormal coagulation response to injury. In severe haemophilia A, Factor VIII level is <1%, often manifesting with spontaneous bleeding into joints. Judicious use of recombinant Factor VIII therapy to maintain adequate levels in the intraoperative, immediate and late post-operative periods, together with adjuvant pro-coagulants, can ensure a safe outcome following surgery. We describe the successful management of one such patient suffering from Marjolin's ulcer of the right gluteal region, who needed wide local excision followed by flap cover. A protocol for management of such patients is also suggested. This is the first such case report from the Indian subcontinent, with only a few such published reports from the West.http://www.thieme-connect.de/DOI/DOI?10.4103/ijps.IJPS_214_16factor viiiflap coverhaemophilia
spellingShingle Narender Manickavachakan
Sunderraj Ellur
Vijay Thomas Mattyoo Joseph
Jonathan Victor
Cecil R. Ross
Flap cover in a patient with severe haemophilia type A
Indian Journal of Plastic Surgery
factor viii
flap cover
haemophilia
title Flap cover in a patient with severe haemophilia type A
title_full Flap cover in a patient with severe haemophilia type A
title_fullStr Flap cover in a patient with severe haemophilia type A
title_full_unstemmed Flap cover in a patient with severe haemophilia type A
title_short Flap cover in a patient with severe haemophilia type A
title_sort flap cover in a patient with severe haemophilia type a
topic factor viii
flap cover
haemophilia
url http://www.thieme-connect.de/DOI/DOI?10.4103/ijps.IJPS_214_16
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AT jonathanvictor flapcoverinapatientwithseverehaemophiliatypea
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