Acquired hemophilia in a 7 year-old girl successfully treate
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Acquired hemophilia should be evaluated in pediatric patients with bleeding and isolated prolonged aPTT. Immunosuppressive treatment should be initiated even in minor bleedings.

A 7 year-old girl presented with recurrent self-limiting epistaxis. Anamnesis revealed mother and male sibling were beta-thalassemia carriers.

Oral tranexamic acid was prescribed after otolaryngologist evaluation was diagnosed with bleeding varicose vessel in her nasal septum. After one week, due to frequent hemorrhagic episodes and asthenia onset, she was admitted once again. Her blood examinations revealed microcytic anemia (hemoglobin 7.9 g/dL, mean corpuscular volume 59.4 fl), and an isolated prolonged aPTT (116 seconds). Iron oral supplement was started, and the patient was discharged.

A few days later, patient was finally admitted to Hematology Oncology Pediatric Department. Blood tests showed increasing hemoglobin levels and reticulocytosis, confirming the iron deficiency and initial response to oral therapy. On the other hand, aPTT prolongation (116 seconds) was confirmed, and did not improve after incubation with mixing test in a 1:1 relation with plasma from a healthy control. Clotting factors concentration revealed almost complete FVIII deficiency (0.7%). Antistreptolysin O titer tested positive (1002 UI/mL).

A week later, the girl was admitted again complaining of intense headache and mental confusion, and presenting at general examination with a vast hematoma, undetectable few days before, extending from her left hip to the knee with walking impairment. Ultrasonography showed patency of the deep venous system, while MRI confirmed the presence of a large inflammatory component of high intensity on STIR, hyperintense in both T2 and FAT SAT with slight peripheral edema, compatible with a muscular hematoma. Her blood tests revealed severe anemia (hemoglobin 5 gr/dL), heart rate was accelerated, and so two emergency red blood cell transfusions were executed. Coagulation assessment revealed 0% of factor VIII activity levels, with the aPTT confirmed at 117 seconds. FVIII Inhibitor assay was positive with high titer (8.9 BU). Clinical, anamnestic, and laboratory diagnostic criteria confirmed acquired hemophilia.

IV administration of rFVIIa was started, the only available bypassing agent, at the dose of 90 µg/kg every 2-3 hours and oral prednisone (1 mg/kg/day). After initial clinical and laboratoristic improvement, with hematoma resolution and increase of hemoglobin level, the time of administration of rFVIIa was gradually prolonged until complete suspension in six days.

During the two weeks of hospitalization, she achieved a complete response, achieved hemostatic control documented by progressively decreasing aPTT count and undetectable inhibitor's titer at a second measure.

Patient was discharged on oral prednisone, tapered until suspension in eight weeks. After 6 months of follow-up, the patient is symptom free at the moment, with normal levels of both aPTT and FVIII activity, and the absence of autoantibodies.

Source: https://onlinelibrary.wiley.com/doi/full/10.1002/ccr3.3588?af=R
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