A 34-year-old man diagnosed with severe malaria in Cuba 25 years ago (his country of origin and resident in Spain in the last 6 years).
She reported no family history of the disease.
During these years he presented two episodes of hemarths in the right knee, treated conservatively, requiring cryoprecipitations.
Before the current episode, the patient was asymptomatic and without home medical treatment.
The patient came to the emergency room of another hospital for abdominal pain, located in hypogastrium, with 3 hours of evolution associated with hematuria.
After performing analytical and radiological tests he was diagnosed with nephritic colic, being discharged with analgesic treatment.
Five hours later, during the micturition, he suffered a syncope with loss of knowledge of few seconds of duration, so he came to the emergency room of our hospital.
On physical examination, the patient was conscious and oriented, with regular general condition and intense mucocutaneous toxicity.
Systolic blood pressure was 70 mmHg and diastolic blood pressure was 37 mmHg, with a heart rate of 61 beats/min. Abdominal examination revealed tender abdomen, painful peritoneal irritation and diffuse signs.
Hematocrit was 20%, hemoglobin 6.7 g/dl, leukocytes 12.270/ul (84% neutrophils) and platelets 358.000/ul.
Prothrombin 100%, TTPA 46%.
An ultrasound showed abundant left subdiaphragmatic fluid, subhepatic fluid and paracolic droplets.
In the vesicorectal space, an oval image with fluid inside was visualized.
It was decided to perform a CAT scan where the same cystic image was observed retro and supravesical, 5 cm in diameter, which seems to depend on an arterial vessel but without detecting its origin.
Having suspected bleeding in a hemophiliac patient, it was decided to perform an immediate transfusion of two solid concentrates, three factor VIII vials and another recombinant factor VII vial.
The administration of factor VIII required laboratory evaluation of anti-factor VIII antibodies that were positive and required, prior to administration, the application of anti-factor VIII inhibitors (4).
After the analytical improvement and due to the persistence of the patient's bad general condition, a suprainfraumbilical midline laparotomy was performed finding hemoperitoneum of approximately 3 clots and red blood cells.
A cystic tumor of 6 cm, retrovesical, of elastic consistency and surface intimately attached to the posterior aspect of the bladder was found.
Rome and exeresis of the tumor were performed.
Subsequently, the rest of the abdominal cavity was reviewed without finding an active bleeding point.
The patient recovered satisfactorily in the ward, remaining afflicted, with haematocrits maintained and decretory debits due to drainage, and was discharged on the tenth postoperative day.
The patient required follow-up visits due to signs and symptoms consistent with factor VIII administration every 12 hours for the first four days.
Pathology reported a cupuliform formation of 6.5 cm in diameter.
The external surface and internal surface showed multiple adhered clots.
The fibrous dome contained moderate inflammatory infiltrate with chronic nonspecific predominance with some hemophagogues.
On the internal surface, he presented mature adipose tissue, in addition to the attached material, which was compatible with hemophilic pseudotumor.
