Cargando…

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 ma...

Descripción completa

Detalles Bibliográficos
Autores principales: Manickavachakan, Narender, Ellur, Sunderraj, Mattyoo Joseph, Vijay Thomas, Victor, Jonathan, Ross, Cecil R.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5770937/
https://www.ncbi.nlm.nih.gov/pubmed/29343899
http://dx.doi.org/10.4103/ijps.IJPS_214_16
Descripción
Sumario: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.