Asian Journal of Transfusion Science
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Year : 2021  |  Volume : 15  |  Issue : 2  |  Page : 237-240

Surgical management of endometriosis in a severe Hemophilia A female patient and the role of transfusion medicine specialist: A case report with review of literature

1 Department of Transfusion Medicine, Kerala institute of Medical Science, Trivandrum, Kerala, India
2 Department of Immunohematology and Blood Transfusion, Kasturba Medical College Manipal, Manipal Academy of Higher Education, Manipal, Karnataka, India
3 Department of Transfusion Medicine, District Hospital, Idukki, Kerala, India
4 Department of Transfusion Medicine, Jubilee Mission Medical College, Thrissur, Kerala, India

Correspondence Address:
Ganesh Mohan
Department of Immunohematology and Blood Transfusion, Kasturba Medical College-Manipal, Manipal Academy of Higher Education, Udupi, Karnataka - 576104
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ajts.ajts_6_21

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Hemophilia A is an X-linked recessive bleeding disorder occurs due to deficiency of factor VIII (FVIII). The disease manifests exclusively in males though it rarely occurs in females due to complex pathophysiological mechanisms. We present a rare case of female hemophilia due to skewed X-inactivation which adversely affected the quality of patient life. She presented with recurrent abdominal pain and was diagnosed with severe endometriosis and underwent total abdominal hysterectomy with left salpingo-oophorectomy and appendicectomy. She was infused recombinant factor VIII both prophylactically and postoperatively as per the World Federation of Hemophilia guidelines. Recombinant Factor VIII was supplemented every 12th hourly and Factor VIII activity levels were monitored daily. She was discharged uneventfully on the postoperative day 21 after screened negative for acquired inhibitors.

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2006 - Asian Journal of Transfusion Science | Published by Wolters Kluwer - Medknow
Online since 10th November, 2006