Proposal of Immunotolerance Induction in Haemophiliacs A with Inhibitors Undergoing to Pressing Elective Surgery by Using FVIII/vWF-enriched Pure Plasmaderivates

Abstract number: P0216

R¹ Musso, D² Cultrera, S³ Motta, C³ La Greca, M¹ Musso, A¹ Cipolla, R² Giustolisi

¹¹Haemophilia and Thrombosis Regional Reference Center, Hematology Dpt, University of Catania, Italy ²²Hematology Dpt., University of Catania, Italy ³³Vth Division of General Surgery, Catania, Italy

To date, an urgent elective surgery in haemophiliacs A (HA) with inhibitor even in those low responders or with long-standing inhibitor (LSI), not undergone in the past to immunotolerance treatment (ITT), remains a big concern. In fact, even if widely advised from physicians is often refused. In our opinion, ITT may be really performed in expectation of a pressing elective surgery. On the basis of our previous positive ITT result in an adolescent HA with LSI-high responder type treated with pure FVIII/VWF-enriched plasma concentrate (Fanhdi®, Grifols, Italy), we here report a further success of ITT in an adult severe haemophiliac (43 years old), with low responder inhibitor (4.7 UB/ml, lasting 15 years) (HCV+ and HIV-) and recurrent hemartroses, undergoing to urgent elective surgery. In August 2003 the patient experienced frequent painful colics for congenital retention of the right testicle in the abdomen. The computered tomography confirmed his pathology. The video-laparoscopic laser-surgery was successfully performed by bolus infusion of Fanhdi (8.000 UI, Grifols, Italy). During the post-operative period the concentrate was infused (2.000 UI, twice/day) for one week. No bleeding was observed and he was discharged after 5 days. The patient enthusiastically continued ITT (Fanhdi, 8.000 UI/week) at his home for 6 months. The inhibitor progressively sloped and the ‘in vivo’ recovery (IVR) was normal (>6 h) 15 days after the operation. At present, the inhibitor remains negative; the IVR is normal. No hemartroses have been seen in these last 16 months; he actually gained his articular motions and remains on therapy with the same concentrate (4.000 UI/week) to have a normal quality of his life. On the basis of recent reports regarding the favourable achievement of ITT by using plasma FVIII/VWF-enriched pure derivates, we suggest that ITT induction with these products can be considered in haemophiliacs with inhibitor undergoing to an urgent elective surgery.