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

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XVIII Congress of the Italian Society for Hemostasis and Thrombosis Research, Rome, Sept. 30-Oct. 3, 2004103Italian patients showed a sporadic risk of infectionwith HIV and a lower prevalence of HCV. As expected,HCV genotypes distribution reflects the sourceof blood products and might influence the naturalhistory of HCV in VWD.PO-019RECURRENT GASTROINTESTINAL BLEEDING IN A PATIENT WITHTYPE 3 VON WILLEBRAND DISEASE AND MULTIPLE MUCOSALVASCULAR ABNORMALITIES: A ROLE FOR PROPHYLAXISWITH FACTOR VIII/VON WILLEBRAND CONCENTRATE ANDOCTREOTIDECoppola A, Cimino E, Nardone G,^ De Simone C,Conca P,* Di Minno M, Sidiropulos M, Albisinni R,Di Minno GRegional Reference Centre for Coagulation Disease,^Chair of Gastroenterology and *Sector of Hepatologyin Internal Medicine, Dep. of Clinical and ExperimentalMedicine, Federico II University, Naples, ItalyThe association between von Willebrand Disease(vWD) and mucosal vascular abnormalities (MVA) ofthe gut is well recognized, often resulting in severebleeding tendency and difficult therapeutical management.We report the case of a 57 yr-old man withtype 3 vWD and a history of recurrent melena sincethe age of 53. Endoscopic examinations revealedmultiple small MVA of the stomach and, two yearslater, of the left large bowel. Bleeding frequency andseverity progressively increased and the patientshowed chronic anemia and hemoglobin in thestools. Electro-coagulation of some actively bleedinglesions and octreotide courses did not significantlyaffect his clinical outcome: during 2002 the patientneeded transfusions (6 packed red cell units) andabout 400.000 IU of factor VIII/von Willebrand concentrates(Haemate P, Aventis Behring) on demand.The administration of Haemate P 40 IU/Kg thriceweekly was then proposed. This regimen was associatedwith improvement of the patient meanhaemoglobin levels and the cessation of clear-cutmelena and red cell transfusions. Total Haemate Punits infused were also reduced by about 15% during2003. Clinical conditions and Haemate P requirementsfurther improved when the patient was regularlycompliant to s.c. octreotide (0.1 mg b.i.d.)administration (he did not tolerate multiple dailyinjections). Prophylaxis with Haemate P is still ongoingwithout any adverse event over a 18-mo. period.Clinical course and pharmacokinetic evaluations ledto administer 40 IU/Kg each 72 hours. Possible vascularcomplications were excluded by careful clinicaland instrumental follow-up, as the patient sufferedfrom arterial hypertension and diabetes mellitus;thrombophilic abnormalities were previouslyexcluded and no signs of coagulation activation weredetected thereafter. Long-term prophylaxis withHaemate P has been showed to be safe and effectivein this patient with severe gastrointestinal bleedingdue to MVA and vWD.PO-020DOES THE COEXISTENCE OF VON WILLEBRAND FACTORDEFICIENCY AND PROTHROMBOTIC GENE POLYMORPHISMSREDUCE THE OCCURRENCE OF THROMBOEMBOLIC EVENTS?Lombardini D, Tufano A, Coppola A, Sidiropulos M,De Simone C, Cerbone AM, Di Minno GRegional Reference Centre for Coagulation Diseases;Department of Clinical and ExperimentalMedicine, "Federico II" University, Naples, ItalyA 28-year-old man was referred to our Centrebecause of a recent post-traumatic deep venousthrombosis of the leg and acute pulmonary embolism.He carried heterozygosity for Factor V Leidenmutation and homozygosity for the thermolablevariant of the 5,10-MTHFR, with moderate hyperhomocysteinemia.His mother, who developed juvenileischemic heart disease in the absence of establishedrisk factors for arterial thrombosis, had a positivefamily history for early-onset arterial thromboticevents. She exhibited, in addition to the geneticabnormalities of the son, heterozygosity for the FIIG20210A variant. Her second son was also studiedwho, at variance with his mother and his brother,did not develop thrombotic events. He exhibited heterozygosityfor the Factor V Leiden mutation,homozygosity for the thermolable variant of the5,10-MTHFR, with moderate hyperhomocysteinemia,and a mild prolonged aPTT. Factor VIII coagulantactivity and von Willebrand factor were both reduced(Factor VIII=65%, and vWF=48%), and the screeningfor Lupus Anticoagulant and Anticardiolipin antibodieswas negative. Negativity for these antibodies,as well as normal FVIII and vWF were found in hismother and brother. Nobody in the family experiencedhemorrhagic events. In a family with a highprevalence of thrombophilic gene abnormalities, thecoexistence of reduced factor VIII and vWF levels islikely to have contributed to modulate the differentphenotypic expression of the siblings.haematologica vol. 89(suppl. n. 8):september 2004
104PostersPO-021EVALUATION OF A NOVEL AUTOMATED,IMMUNOTURBIDIMETRIC ASSAY FOR VON WILLEBRANDFACTOR ACTIVITY DETECTIONVaccarino A,° Montaruli B,° Valpreda A,^ Agnes C,°Borchiellini A,^ Scirelli T,^ Saitta M,* Bazzan M°°Department of Haematology and CoagulationDisorders and *Laboratory Analysis – OspedaleEvangelico Valdese; ^Divisione Ematologiadell’ Università, Azienda Ospedaliera San GiovanniBattista; Turin, ItalyPatients with von Willebrand’s disease (vWD) mayhave normal levels of von Willebrand factor (vWF)antigen. It is therefore very important to measurenot only the antigen concentration but also the vWFactivity. The standard assay detect the ristocetincofactor activity (vWFR:Co) by measuring the abilityof patient’s plasma to induce agglutination offormaldehyde-fixed normal platelets in the presenceof a fixed amount of ristocetin. This can be measuredusing a platelet aggregometer, a slide technique or,more recently, photo-optical coagulometer. However,vWFR:Co assay standardization, due to plateletpreparation and in some cases to the agglutinationdetection, is difficult and can affect test reproducibility.We assayed the performance characteristicsof a new commercial and fully automated assayalternative to vWFR:Co for the quantification of vWFactivity (von Willebrand Factor Activity, HemosiIL, IL,USA). This new assay is a latex particle enhancedimmunoturbidimetric test which determines the vWFactivity by measuring the increase of turbidity producedby the agglutination of the latex reagent. Aspecific anti-vWF monoclonal antibody adsorbedonto latex reagent, directed against the plateletbinding site of vWF (Glycoprotein Ib receptor), reactswith the vWF of patients plasma. The degree ofagglutination is directly proportional to the vWFactivity in the sample and is determined by measuringthe decrease of transmitted light caused by theaggregates. Forty-three samples were analysed:12normal controls, 20 congenital vWD patients (3 postdesmopressin somministration) and 3 acquired vWDpatients; moreover, in order to evaluate the specificityof this new assay, we studied patients with differentcoagulation abnormalities: 1 patient treatedwith low molecular weight heparin therapy (LMWH),6 patients with Lupus Anticoagulant (LA) and 1patient with factor VIII:C deficiency. VWF activitywas assayed by vWFR:Co aggregometric method(Ristocetin Cofactor Assay, Helena) on PACKS’ 4(Helena) and by a recently developed immunoturbidimetricmethod (von Willebrand Factor Activity, IL,USA) on the automated coagulomter (ACL9000, IL).In addition we measured platelets functionality byusing PFA-100 (Dade Behring) and vWF antigen byELISA (Asserachrom vWF, Roche) and immuno-turbidimetricmethod on photo-optical coagulometer(von Willebrand Factor, IL, USA) in all patients andcontrols. Good intra and inter assay precisions wereobserved with the IL vWF activity assay using normalplasma pool: CV = 5.7% and CV = 8.6% respectively.All the congenital and acquired vWD patients aswell as normal controls and patients with coagulationabnormalities different from vWD were correctlydifferentiated by this new vWF activity assay. Allpatients treated with DDAVP showed a two/threefold increase of vWF activity with this new coagulometricassay. The method comparison plots showeda good correlation of the IL vWF activity assay withthe aggregometric vWFR:Co method (r = 0.95, PassingBablok regression y = 1.1202 × + 7.9956) and ofthe latex immunoassay for the determination of thevon Willebrand antigen with the ELISA method (r =0,96 , Passing Bablok regression y = 1.0970 × –1.6614). Interestingly one patient with congenitalvWD and contemporary LA autoantibodies was foundto be normal for IL vWF activity on the undilutedplasma, this sample after 1:100 dilution showed 28%of IL vWF activity. Our preliminary data show thatthis new automated immunoturbidimetric IL vWFactivity assay is useful for the diagnosis of congenitaland acquired vWD, its easy and rapid performancesuggest its routine application in combinationwith automated latex immunoassay for the quantitativedetermination of vWF antigen for the vWDdiagnosis. Further studies are needed about plasmaticconditions which may give interferences inthis new vWF activity assay.PO-022SENSITIVITY AND SPECIFICITY OF PFA-100 ® VERSUSBLEEDING TIME IN THE DIAGNOSIS OF TYPE 1 VONWILLEBRAND DISEASECastaman G,* Tosetto A,* Bernardi M,*Bertoncello K,* Peake I,° Goodeve A,° Federici AB, +Batlle J,^ Meyer D,** Eikenboom J,°°Schneppenheim R, ++ Ingerslev J,^^ Vorlova Z,***Lethagen S,°°° Pasi J, +++ Hill F,^^^ Rodeghiero F**San Bortolo Hospital, Vicenza, Italy; °University ofSheffield, United Kingdom; + University of Milan,Italy; ^Hospital Teresa Herrera, Spain; **INSERM,France; °°Leiden University Medical Center, Netherlands;++ University Children Hospital, Hamburg,Germany; ^^University Hospital Skejby, Denmark;***Institute of Hematology and Blood Transfusion,Prague, Czech Republic; °°°University of Lund, Sweden;+++ Leicester Royal Infirmary, UK; ^^^BirminghamChildren Hospital, UKhaematologica vol. 89(suppl. n. 8):september 2004
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haematologicaThe origin and power o
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