Haematologica 2004;89: supplement no. 8 - Supplements ...

190PostersAt the last follow-up, 11 patients with severe hemophilia,treated on demand, showed a mean orthopedicjoint score of 4 and a mean number of hemartrhosesper year of 2.3; 9 patients with moderatehaemophilia showed a mean orthopedic joint scoreof 1.1 and a mean number of haemartrhoses per yearof 1.5. Sixteen patients began prophylaxis at a medianage of 6.5 (range 2.1-15.6). Nine out of 16received prophylaxis for a period 45 weeks per year.Before starting prophylaxis and at last control, meanorthopaedic joint score was 3.9 and 0.66 respectively(p=0.0069), and mean number of haemartrhosesper year was 3.5 and 0.47 respectively (p=0.00035).Seven/16 received prophylaxis for a period < 45weeks per year; they showed at last control a meanorthopaedic joint score of 1.2 and a mean number ofhaemarthroses per year of 2.5. Two patients are notevaluable because in immunotolerance program.Prophylaxis in severe haemophilia patients results ina significantly better orthopaedic outcome than theone observed in severe and moderate hemophiliapatients on demand treatment. In order to obtaingreatest efficacy, patients on prophylaxis must betreated without discontinuation.PO-188KIDNEY AND BLADDER STONES IN HAEMOPHILIA PATIENTSMusso R, Cultrera D,* Chiarenza A,* D'Arpa S,Cipolla N, Burgio N, Giustolisi R*Haemophilia and Thrombosis Regional ReferenceCenter, *Division of Clinical Haematology, Universityof Catania, ItalyTo date, there are few data about kidney stones(KS) and bladder stones (BS) in haemophilia patientsand it is still unknown if these complications fall inthe general population prevalence or should be considereda new emergent concern in haemophilia. Westudied our large cohort of haemophiliacs to collectin an uniform way clinical data regarding KS and BSprevalence. One hundred and twenty two [males 109,females 15 including severe Factor XI:C deficiency(n=8) and obligate symptomatic carriers (n=5)patients, with a mean age 27.07 years] were followed.KS (n=16) and BS (n=7) were found in 19 (13males, 6 females) for a total prevalence of 15.8% vs5-10% in the general population. We observed ayounger age (23.4 years) in hemophiliac patientscompared with normal population (peak of frequencybetween 40-70 years). From our observation, it isnecessary to evaluate a larger haemophilia patientnumber of different Centres to confirm our data. Amore dept research on this pathology will be usefulto know which other factors among the anamnesticand/or recurrent hematuria, substitution therapiesor antiphibrinolitics agents may play a key role inthe pathogenesis of these complications. If thesedata would be confirm, a new chapter will be addedin hemophilia related pathologies.PO-189IN VIVO RECOVERY (IVR), EFFICACY AND SAFETY OFSOLVENT/DETERGENT (S/D) TREATED PLASMA IN PATIENTSWITH RARE INHERITED COAGULATION DISORDERS (RICDs)Mancuso ME,* Santagostino E,* Morfini M,^Schiavoni M,° Tagliaferri A, # Barillari G,°°Mannucci PM**Angelo Bianchi Bonomi Hemophilia and ThrombosisCenter, IRCCS Maggiore Hospital, Milan, Italy;^Hemophilia Center, Careggi Hospital, Florence,Italy; °Hemophilia & Thrombosis Center, PoliclinicoHospital, Bari, Italy; # Hemophilia Center, ParmaHospital, Parma, Italy; °°Hemophilia Center, S. Mariadella Misericordia Hospital, Udine, ItalyVirus-inactivated concentrates are not availablefor most RICDs and virus-inactivated plasma is recommendedas therapeutic alternative. This prospectivemulticenter study evaluated the efficacy andsafety of S/D plasma (Octaplas, Kedrion, Italy), theIVR of the deficient factors and the dose regimensrequired for RICDs. Seven-teen patients wereenrolled (age: 13-65 yr; M/F: 6/11): 8 had severedeficiencies (1 afibrinogenemia, 2 FV, 1 FX, 4 FXI)and 9 had mild deficiencies (2 FV:20-27%; 6 FV/FVI-II:12-20/6-47%; 1 FXI:30%). Seronegative patientsfor HIV, HCV, HBV, HAV and parvovirus B19 were 17,14, 7, 5, and 1, respectively. Reasons for treatment:delivery (2), minor surgery (5) and major surgery (10).Octaplas (6-29 mL/Kg) was given at 6-96h intervals,according to factor half-lives and clinical features fora mean treatment duration of 5 days. FVIII and/orDDAVP were associated for replacement of FV/FVIIIdeficiency. Mean IVRs: FV 1.8 dL/Kg (0.4-2.9), FVIII0.8 dL/Kg (0.4-1.5), FXI 1.3 dL/Kg (0.6-1.8), FX 1.8dL/Kg and fibrinogen 1.7 dL/Kg. Two perioperativehaemorrhages, in a patient maintaining FXI levels20-41% and in a patient with FV 43%, were controlledby surgical haemostasis and further Octaplasadministrations. Postoperative bleeding in a FV/FVI-II deficient patient occurred when FVIII was 18% andit was controlled by giving FVIII instead of DDAVP.Rash during Octaplas infusion occurred in 1/3patients who had had reactions to FFP. No viral seroconversionwas observed during one-year follow-up.Octaplas was safe and effective in patients withRICDs and it should be preferred when virus-inactivatedconcentrates are not available.haematologica vol. 89(suppl. n. 8):september 2004