A 54-year-old male patient with no family history of bleeding diathesis and personal history of facial dermatitis with intermittent steroidal treatment for 20 years with bilateral cataract was operated.
In June 2000, the patient underwent radical prostatectomy due to localized adenocarcinoma proposis, later controls of antigen proposis specific range (APE).
In February 2003, the patient consulted for a one-year history of recurrent epistaxis and spontaneous appearance of ecchymosis in the trunk and extremities and minor trauma.
In the initial evaluation, prolonged activated partial thromboplastin time (aPTT) was detected in more than twice the normal control, so it was referred for evaluation.
Clinically, a patient was in good general condition, reported no specific discomfort, no history of weight loss or night sweats.
Physical examination revealed large ecchymosis in the lower limbs.
General tests were normal: hemogram, ESR, biochemical profile, liver profile, lactic dehydrogenase.
Hemostasis study revealed:
- Prothrombin time (PT): 76% (Normal: 70-100%). - Activated partial thromboplastin: 73 seconds.
Factor IX: 55% (Normalml: 50-100%). - Factor VIII (FVIII: C): 3% (Normal: 50-150%). - FVW:
With these results, he proposed the diagnosis of AHA with high titer inhibitor and the etiological investigation was initiated.
Prostate cancer activity (computed axial tomography of thorax, abdomen and pelvis, normal bone scintigraphy and PSA) was not demonstrated.
Clinical and laboratory rheumatologic evaluation was negative (ANA, anti DNA antibodies, rheumatic factor, anticardiolipin antibodies, and lupus anticoagulant).
Due to previous steroid toxicity, treatment with oral cyclophosphamide 100 mg daily was initiated maintaining serial clinical and laboratory controls (TTPa, inhibitor titer).
Its control during seven months of treatment demonstrated the persistence of hemorrhoids, without observing a significant decrease in the titer inhibitor, or secondary toxicity secondary to radiation therapy.
The lack of response and the recent publication of the successful use of rituximab in HAA8 were discussed with the patient this therapeutic option and it was decided to treat this antibody.
In October 2003, she received a cycle of rituximab at the recommended dose (375 mg/m2 for four weekly doses).
Before receiving the fourth dose, the patient consulted for left inguinal pain, physical examination showed limited hip flexion, so the diagnosis of psoas muscle hematoma was proposed.
Laboratory study at that time revealed FVIII: C 4% and inhibitor titer 110 BU.
Abdominal computed tomography (CT) revealed a hematoma of the left psoas muscle (volume 70 mi).
She was treated with absolute rest and analgesia, with a rapid decrease in symptoms, confirming a control CT scan the regression of the hematoma.
In the months following the rituximab cycle, a progressive decrease in inhibitor associated with normalization of FVIII: C levels was observed, disappearing the inhibitor 5 months after rituximab treatment.
Clinically, complete remission of hemophiliac symptomatology was observed.
In subsequent controls, until January 2010, it remained in clinical and laboratory remission (67 months of complete remission).
During this period of remission, the patient underwent inguinal herniorrhaphy and a laparoscopic cholecystectomy without complications.
