Dear Editor,
The main defect in patients with haemophilia A is impairment of the coagulation factor FVIII secretion and/or activity from the sinusoidal cells of the liver. This is due to the specific mutation in the factor VIII gene located in the X chromosome [1]. Haemophilia A is classified as severe, moderate, or mild haemophilia in the clinic based on factor VIII protein levels: Moderate haemophilia A is defined by factor VIII (FVIII) levels between 1 and 5% [2]. These classifications generally suggest that severe haemophilias have higher risk of bleeding [3]. Haemarthrosis and haematoma are the most prevalent features of the disease. In addition, moderate haemophilia may be asymptomatic and diagnosed incidentally at the time of surgery or trauma, which can result in serious complications. We present a spontaneous retrovesical haematoma that developed in a 25-year-old male patient who had moderate haemophilia A but did not show any history of severe bleeding.
A 25-year-old male patient diagnosed with moderate haemophilia A and the replacement of the factor at the time of injury or bleeding, was admitted to the emergency department with syncope and epigastric abdominal pain. The patient had no history of abdominal trauma. Physical examination revealed tenderness over the right lower quadrant of the abdomen. Laboratory results revealed anaemia (haemoglobin 9 g/dL). Ultrasonography (USG) of the abdomen revealed no abnormalities. Pelvic magnetic resonance imaging (MRI) determined a haematoma measuring approximately 7 × 6 cm in a retrovesical location (Fig. 1). FVIII was administered randomly to the patient at a dose of 25 IU/kg every 12 h for 10 days. Plasma factor VIII level monitoring could not be done during the FVIII replacement therapy. The clinical signs started to improve with FVIII treatment. The factor treatment intervals were increased during follow-up. After 3 weeks, the haematoma volume was shown to be significantly decreased on a pelvic MRI (Fig. 2). A pelvic MRI performed after 10 weeks showed no haematoma.
Fig. 1.
The MRI of abdomen, showing a retrovesical haematoma at first arrival
Fig. 2.
The MRI of abdomen, showing a regression in haematoma under rFVIII treatment
To our knowledge, this is the first reported cases of spontaneous retrovesical haematoma in haemophilia. The most often reported cases of retrovesical haematomas are complications occuring during gynecological [4] and urogenital surgery [5].
Diagnosis of iliopelvic pain may also be difficult in haemophilia patients. Hip haemarthrosis, iliopsosas haematoma and acute digestive tract disorder (including appendicitis or diverticulitis) should be considered as priorities in this area. However, unusual aetiologies should not be excluded. As seen in our case, USG was observed to be useless in the diagnosis of retrovesical haematomas. MRI definitely provides the best diagnostic capabilities and should be the method of choice where possible.
Factor VIII (FVIII) replacement therapy is essential in curative and preventive management of bleeding complications. Most haematomas regress with a conservative approach and factor replacement. Our case was also treated successfully with factor treatment.
In conclusion, retrovesical haematoma should be considered as one diagnosis for iliopelvic pain in haemophilia. It should also be kept in mind that haematomas may occur in atypical locations regardless of being classified as hemophilia.
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Conflict of interest
The authors declare that they have no conflict of interest.
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Informed signed written consent was taken from the patient involved.
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References
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