Clin Med Rev Case Rep ISSN: 2378-3656

• Abstract
• Teaching Points
• Introduction
• Case
• Discussion
• Precise
• Figure 1
• References

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Clinical Medical Reviews and Case Reports

¹Professor of Obstetrics and Gynecology, University of Central Florida College of Medicine, Florida, USA
²Ob/Gyn Private practice in Melbourne, Florida, USA
³Health First Gynecologic Oncology, Florida, USA

Abstract

Background: Hemophilia A is exceedingly rare in females. Reported here is a case of hemoperitoneum secondary to an endometrioma, which mimicked ovarian cancer on imaging.

Case: A 39 year old with a known complex adnexal mass and a vague history of hemophilia presented with abdominal pain. Imaging was suspicious for ovarian cancer. The patient was discharged with a plan for elective surgery. She represented 2 days later with a massive hemoperitoneum and coagulopathy. Emergent laparotomy with salpingo-oophorectomy for an endometrioma resolved the problem. Subsequent review of records confirmed hemophilia A.

Conclusion: A rare bleeding disorder may be associated with hemoperitoneum in a woman with a hemorrhagic ovarian tumor. The rarity of the condition may lead to initial confusion regarding the correct diagnosis.

Teaching Points

• Although exceedingly rare, hemophilia A does occur in females.

• Hemophilia A in a female may be associated with significant hemorrhage from a hemorrhagic ovarian tumor.

• The diagnosis of ovarian hemorrhage in a patient with hemophilia A may initially be difficult to arrive at, in part due to the rarity of the condition.

Introduction

Hemophilia A is a congenital X-linked recessive bleeding disorder due to factor VIII deficiency. It is exceedingly rare in females. The association of the most common bleeding disorder (Von Willebrand Disease) and hemoperitoneum secondary to a hemorrhagic corpus luteum is well known [1]. Most women with Hemophilia A are asymptomatic but scattered moderate and severe cases have been described [2]. In addition, there have been isolated reports of bleeding disorders associated with endometriosis [3]. Described here is what we believe to be the first case of hemoperitoneum secondary to an endometrioma associated with Hemophilia A.

Case

A 39 year old woman who was followed for several months with a benign appearing complex adnexal mass presented to the emergency department with abdominal pain. She gave a vague history of hemophilia with "easy bleeding" and one uneventful vaginal delivery. The hemoglobin level was 8.8 g/dl. CT scan was interpreted as ascites and a complex 7 cm pelvic mass (Figure 1). The patient was transferred to Holmes Regional Medical Center, seen by gynecologic oncology and discharged home with a plan to be booked for elective surgery. Two days later the patient presented with pain, a dramatic increase in the amount of peritoneal fluid seen on imaging, and hemoglobin of 4.5 g/dl. INR on admission was 4.9, dropping to 1.1 after 1 unit of fresh frozen plasma, 1 unit of prothrombin complex concentrate and 3 units of blood. Emergency laparotomy revealed a hemoperitoneum of approximately 2500 cc and a right ovarian endometrioma, which was removed. Recovery was uncomplicated. Subsequent review of hematology records confirmed a diagnosis of Hemophilia A. Genetic testing has not been performed to date.

Discussion

The rarity of Hemophilia A in a female and the insidious presentation of this case led to concern for possible ovarian cancer. In retrospect, consideration should have been given to hemorrhage from a benign tumor, which may have prevented acute massive hemoperitoneum. In women with a bleeding disorder, a functioning ovary and apparent free peritoneal fluid, consideration should be given to hemoperitoneum.

Precise

A woman with hemophila A and suspicious imaging ultimately developed massive hemoperitoneum secondary to an endometrioma.

References

1. (2013) Von Willebrand Disease in Women. ACOG Committee Opinion No, 580.
   


2. Di Michelle DM, GibbC, Lefkowitz JM, Ni Q, Gerber LM (2014) Severe and moderate hemophilia A and B in US females. Hemophilia 20: e136-e143.
   


3. Valizadeh N, Bromand F, Ayatollahi H (2013) Association between a bleeding tendency and endometriosis (presentation of two cases). J Applied Hematol 4: 118-120.