Abstract Number: PB0647
Meeting: ISTH 2021 Congress
Background: The incidence of inhibitors in NS-HA was estimated between 3-13% but certain F8 genotypes have been identified as associates with higher inhibition.There are limited data about optimal therapeutic approach to eradicate inhibitors in NS-HA patients.
Aims: Explain our strategy for suppression of inhibitor antiF8 in mild HA patient who developed an inhibitor 2 months after treatment of spontaneous epidural spinal hematoma that required intensive replacement with F8 Concentrate (CF8) (ReFacto®) and Desmopressin, and decompressive laminectomy
Methods: Patient, seventeen, Afro-Caucasian, mild HA (F8: C 15 %). The mutation is c.6301 C> G (exon 22) that involves change from aa to p. His2101Asp (fC1 domain). Respondent to Desmopressin. Two months after hematoma and having carried out negative inhibitor controls (2), he went to Urgencies for pain in right iliac fossa of 4 days; Abdominal echography showed retroperitoneal hematoma; Analytically, aPTT 2.13 (0.7-1.3) was observed. For a longer aPTT than usual we repeated coagulation (Table 1) and showed HIGH-titer inhibitor antiF8 (12,8 Bethesda Units, uBT).
Results: Hematoma was treated with Novoseven®, 7 days. We began ITI (Induction to Inmuno-Tolerance) at the same time with Immunosuppression (IS) (Prednisone 1 mg / Kg / day, 5 weeks and AntiCD20 Antibody 375 mg / m2 / weekly , 4 weeks). 1 month after AntiCD20, a negative inhibitor titer (<0.6 uBT) was observed. Continuous progressive decrease of Prednisone (10 mg by week) until suspension (2 months). He recovered the response to Desmopressin (Table 1). We made pharmacokinetic study with 30 IU/Kg CF8 (Fandhi®)(WAPPS Hemo®), to certify the success: recovery 2.25//half life 8,25 hours.
Conclusions: This is succesfully ITI in NS-HA with IS. Inhibitors in NS-HA are a relevant clinical problem being imperative to learn more about treatment regimens aimed at eradicating it. IS seems a valid option, as reported by Kempton in a cohort of 32 patients.
To cite this abstract in AMA style:Lopez MR, Lopez Ansoar E, Dominguez Muñiz O, Nieto Vazquez A, Albo Lopez C. Inhibitors in Non Severe Haemophilia A (NS-HA): A Case Report [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 1). https://abstracts.isth.org/abstract/inhibitors-in-non-severe-haemophilia-a-ns-ha-a-case-report/. Accessed September 24, 2021.
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