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O13 Ten years follow up of hereditary hemorrhagic patients in the dental practice
Oral presentations, ISH EAD 2007, Budapest, Hungary, 29 August frequency of mucocutaneous bleeds and serious gynecological pathology in females with severe FVII deficiency. Evaluation of 58 pts with dysFBG (FBG:coag//FBG:Ag 0.66/2.8 g/L) showed 28 (48%) asymptomatic individuals, 27 (47%) and 2 (3.4%) have mild bleeding symptoms and a history of thrombosis, respectively. Nine (8%) of 107 surgeries performed in 33 pts w/o FBG prophylaxis were complicated with bleeding. Gene analysis performed in 26 pts revealed heterozygous mutation Aa Arg16 gHis in FGA exon 2 in 3 families and a novel heterozygous mutation Aa Gly13 gGlu in FGA exon 2 in 2 families. Six out of 14 pts with hypo-FBG (FBG coag/Ag 1.1/1.2 g/L) had mild bleeding tendency, 8 (22%) out of 36 surgeries w/o replacement were complicated with bleeding. A heterozygous novel mutation Trp3 Stop was identified in FGG exon 1 in 3 families (FBG:coag 1.1 g/L) and heterozygous mutation Trp253Cys in FGG exon 7 (FBG:coag/Ag levels of Fibrinogen Bratislava) in one pt with serious 0.7/0.8 g/L postpartum bleeding. Conclusion: The clinical manifestation of the rare bleeding disorders is often variable. The gene analysis may be of value for genetic counselling, however, further studies are warranted to stipulate the usefulness of gene defect identification for the prediction of clinical phenotype. O13 Ten years follow up of hereditary hemorrhagic patients in the dental practice S.P. Makris *, M.P. Makris, A. Papadopoulos, P.E. Makris. Haemostasis and Thrombosis Unit, Aristotle University of Thessaloniki, Greece The last ten years we provided dental care in a group of 160 patients with hereditary haemorrhagic disease in order to change the attitude of these patients concerning dentist and the oral cavity problems. We applied the follow steps: (i) Regular periodic preventive control of the oral cavity (control teeth cleaning, guidance of the patient); (ii) early care of dental problems in order to avoid tooth loss (common treatment in the past); (iii) bloodless confrontation of the mainly bloody interventions (teeth extraction excision of cysts, ectomy of the apex) and (iv) proper measures during the apoptosis of the deciduous teeth. Material: During the last ten years we applied a follow up in 160 patients with haemorrhagic hereditary diseases (hemophilia A and B and von Willebrand’s disease). In 65 patients, 213 teeth extractions were realized and 15 minor surgical cases. Concerning severe haemophiliacs we administered 40 units/kg BW of recombinant factors. All the previous treatment protocols were accompanied by strenuous measures of local haemostasis (we replaced surgical with collagen fleece, splint with fibrin glue and then with pressing tampon due to problems that occurred). Of course important was the use of 10% tranexamic acid solution as local anti-fibrinolytic treatment. Results: In these ten years that we follow up this group of patients a great number of dental actions were performed (250 tooth cleanings and scaling, 353 tooth fillings, 57 endodontic treatments, 213 teeth extractions, 15 minor surgical interventions and 85 deciduous teeth extractions). Conclusion: By avoiding haemorrhagic complications and providing painless conditions of treatment we restored the confidence in the dentist’s face and reassured the acceptance of preventive dental measures.
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Blood transfusion, Friday 31 August 2007 O14 RhD positive or negative? Partial D antigen determination using gel card technique by DIAGAST D Screen system Z. Csernus *, K. Siba, V. P´ aszthy, I. Nemes. NBTS Regional Blood Transfusion Center P´ ecs, Hungary Introduction: Rh(D) typing is an important part of pretransfusion testing, because the D antigen is highly immunogenic. There is about a 70 80% chance that an Rh(D) negative recipient will produce anti-D antibodies following the transfusion of D positive red cells. Ambiguous anti-D reactions are caused by quantitative (weak-D) or qualitative (partial-D) deviations from normal D antigen expression. Donors showing a weak-D or partial-D phenotype are able to immunise Rh(D) negative recipients, but carriers of weak D type may be transfused with Rh(D) positive blood products since they will not produce anti-D. The purpose of our investigation was to determine the frequency of partial- and weak-D phenotypes among blood donors, patients and pregnant women showing ambiguous anti-D reactions. Materials and Methods: We examined a total of 20,097 native blood samples of donors, patients and pregnant women using the microplate technique. 16,170 donor samples were evaluated on an Olympus automated system using Olymp TOTEM (P3x61, P3x21223B10 IgM, P3x290, P3x35 IgG) and anti-D (RH1)P3x61 IgM and Biotest P3x255 13 G8/P3x61/P3x234 IgM monoclonal antibodies. 5,806 patient samples were typed manually using BIOTEST Erytype ABO + D microplates with monoclonal anti-D BS226, BS232 and anti-CDE P3x25513, G8 P3x61/P3x234. using by the indirect antiglobulin tube test (IAT test). All samples with negative or uncertain (weak) reactions were re-evaluated by RhD indirect Coombs test with Seraclone anti-D (RH1) blend monoclonal antibodies (IgG BS 221 and H41 11B7 and IgM BS 232). We performed a partial D screening in all samples showing weak (+) to strong (+++) positive reactions by means of DIAGAST D Screen haemagglutination tests in Scangel cards. The system is able to indentify 11 different partial D categories, and is also suitable for the verification of the weak-D phenotype. Results: Among the samples examined 93 (0.46%) showed an ambiguous reaction with the D antibody. We found the gel card based haemagglutination tests are easy to evaluate, and the system gave definite results. The gel card system requires half the amount of antibodies compared to the test tube method. Of the 93 ambiguous reactions 40 (43%) showed a weak-D character, and the remaining 53 (57%) belonged to one of the following partial-D categories: II (14%), IIIa, IIIb, IIIc (21.5%), VI (8.6%), VII/HMi (12.9%). Sampes with partial-D expression showed the following phenotypes: 68% Cce, 4% cEe, 2% ce, 9% CE. The AB0 distribution were as follows: A 31%, B 16%, 0 30%, AB 9%. Nearly 50% of the ambiguous blood samples exhibited a weak-D character that would place them into the RhD positive category. These patients would be able to receive RhD positive blood products, and the women with this phenotype would not require the administration of anti-D profylaxis during and after pregnancy. Conclusion: The DIAGAST D-screen system is a cost effective and reliable technique for routine use in gel cards. The use of this system helps to avoid the unnecessary administration of RhD negative units to weak D positive blood recipients, and the unnecessary anti-D prophylaxis to weak-D carrier pregnant women. In conclusion this method helps to identify those clinically relevant partial D variants that carry a potential risk for the development of anti-D.
2 September 2007
S71
Blood transfusion, Friday 31 August 2007 O14 RhD positive or negative? Partial D antigen determination using gel card technique by DIAGAST D Screen system Z. Csernus *, K. Siba, V. P´ aszthy, I. Nemes. NBTS Regional Blood Transfusion Center P´ ecs, Hungary Introduction: Rh(D) typing is an important part of pretransfusion testing, because the D antigen is highly immunogenic. There is about a 70 80% chance that an Rh(D) negative recipient will produce anti-D antibodies following the transfusion of D positive red cells. Ambiguous anti-D reactions are caused by quantitative (weak-D) or qualitative (partial-D) deviations from normal D antigen expression. Donors showing a weak-D or partial-D phenotype are able to immunise Rh(D) negative recipients, but carriers of weak D type may be transfused with Rh(D) positive blood products since they will not produce anti-D. The purpose of our investigation was to determine the frequency of partial- and weak-D phenotypes among blood donors, patients and pregnant women showing ambiguous anti-D reactions. Materials and Methods: We examined a total of 20,097 native blood samples of donors, patients and pregnant women using the microplate technique. 16,170 donor samples were evaluated on an Olympus automated system using Olymp TOTEM (P3x61, P3x21223B10 IgM, P3x290, P3x35 IgG) and anti-D (RH1)P3x61 IgM and Biotest P3x255 13 G8/P3x61/P3x234 IgM monoclonal antibodies. 5,806 patient samples were typed manually using BIOTEST Erytype ABO + D microplates with monoclonal anti-D BS226, BS232 and anti-CDE P3x25513, G8 P3x61/P3x234. using by the indirect antiglobulin tube test (IAT test). All samples with negative or uncertain (weak) reactions were re-evaluated by RhD indirect Coombs test with Seraclone anti-D (RH1) blend monoclonal antibodies (IgG BS 221 and H41 11B7 and IgM BS 232). We performed a partial D screening in all samples showing weak (+) to strong (+++) positive reactions by means of DIAGAST D Screen haemagglutination tests in Scangel cards. The system is able to indentify 11 different partial D categories, and is also suitable for the verification of the weak-D phenotype. Results: Among the samples examined 93 (0.46%) showed an ambiguous reaction with the D antibody. We found the gel card based haemagglutination tests are easy to evaluate, and the system gave definite results. The gel card system requires half the amount of antibodies compared to the test tube method. Of the 93 ambiguous reactions 40 (43%) showed a weak-D character, and the remaining 53 (57%) belonged to one of the following partial-D categories: II (14%), IIIa, IIIb, IIIc (21.5%), VI (8.6%), VII/HMi (12.9%). Sampes with partial-D expression showed the following phenotypes: 68% Cce, 4% cEe, 2% ce, 9% CE. The AB0 distribution were as follows: A 31%, B 16%, 0 30%, AB 9%. Nearly 50% of the ambiguous blood samples exhibited a weak-D character that would place them into the RhD positive category. These patients would be able to receive RhD positive blood products, and the women with this phenotype would not require the administration of anti-D profylaxis during and after pregnancy. Conclusion: The DIAGAST D-screen system is a cost effective and reliable technique for routine use in gel cards. The use of this system helps to avoid the unnecessary administration of RhD negative units to weak D positive blood recipients, and the unnecessary anti-D prophylaxis to weak-D carrier pregnant women. In conclusion this method helps to identify those clinically relevant partial D variants that carry a potential risk for the development of anti-D.