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P046 The use of rotation thrombelastography in familial thrombophilia
Posters, ISH EAD 2007, Budapest, Hungary, 29 August Surgical treatment was performed in 5 pts with intracranial (4 subdural, 1 intraventricular), and in 3 pts with abdominal bleeding by laparotomy. rFVIIa was used as last possibility for uncontrolled bleeding in 3 patients with acute leukaemia too. Results: Haemotasis was obtained in all patients; 6 pts were successfully treated surgically (4 subdural, 1 intraventricular and 1 GI bleeding). Conclusion: Therapy of life-threatening bleeding in haemophiliacs is now improved. rFVIIa is haemostatic agent also very useful in other haematological diseases, as leukaemia and thrombocytopenia. P046 The use of rotation thrombelastography in familial thrombophilia ´ . Nagy, H. Losonczy. University O. T´ oth *, N. Lima, M. D´ avid, A of P´ ecs, Ist Dept. of Internal Medicine, P´ ecs, Hungary Introduction: Thrombelastography is a global haemostasis assay, suitable for the monitoring of the coagulability of whole blood. Its main advantages are that the examination of whole native or anticoagulated blood does not require further preparation steps, and gives immediate information on the two main parts of haemostasis, the plasmatic and corpuscular ones. Materials and Methods: A thrombelastograph based on rotation thrombelastography was used in our haemostasis laboratory. The native and anticoagulated (0.129 M trinatrium-citrate) whole blood of 41 healthy volunteers and 55 patients with thrombotic anamnesis (TE) were examined. Four types of tests were performed: nNATEG (native whole blood), NATEG (recalcinated anticoagulated whole blood), INTEG (addition of contact coagulation activator) and EXTEG (addition of tissue factor). Results: In the group of patients with thrombotic anamnesis the clotting time (INTEG: 129.0±33 vs. 145.8±38.6; data are mean±SD for TE patients vs. control subjects) and the clot formation time (INTEG: 66.0±21 vs. 75.3±20.3; EXTEG: 72.4±17.4 vs. 80.4±18.3) were significantly shorter, the maximal amplitude (INTEG: 62.9±5.1 vs. 60.8±4.1; EXTEG: 62.4±4.8 vs. 59.3±4.5) and the alfa angle (INTEG: 77.2±3.9 vs. 75.2±3.9; EXTEG: 76.9±2.9 vs. 74.4±3.35) were significantly higher when compared to the control group, which indicates hypercoagulability. We could not detect any significant differences in the results between the thromboembolic patients with and without thrombophilia. Conclusion: Rotation thrombelastography differentiates between normal controls and TE patients, but it did not prove to be useful in diagnosing familial thrombophilia in our experiments. P047 Acquired dysfibrinogenemia associated with multiple myeloma R. Kotl´ ın *, A. Sobotkov´ a, J. Suttnar, P. Salaj, T. Riedel, J.E. Dyr. Department of Biochemistry, Institute of Hematology and Blood Transfusion, Praha, Czech Republic Introduction: Fibrinogen, a key molecule of the blood coagulation, is a 340 kDa glycoprotein which is synthesized in the liver as a homodimeric molecule containing two sets of three different polypeptide chains (Aa, Bb and g). Multiple myeloma is a plasma-cell neoplasm that is characterised by skeletal destruction, renal failure, anemia and hypercalcemia. Paraproteins are the result of clonal B-cell or plasma cell proliferation of a malignant, premalignant or nonmalignant nature. Monoclonal proteins may consist of intact immunoglobulin molecules or of heavy or light chains only. Paraproteins are able to inhibit coagulation by acting on fibrin polymerization or platelets. Abnormal coagulation properties indicative of dysfibrinogen were found in the plasma of a 72year old male with multiple myeloma (IgG, kappa, stage IIIA).
2 September 2007
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Methods: The cause of abnormal coagulation results was determined using fibrin polymerisation measurement, fibrinogen clottability measurement, sodium dodecyl sulphate polyacrylamide gel electrophoresis (SDS-PAGE), Western blotting, SEM, DNA sequencing, MALDI-TOF MS and ELISA. Results: The patient admitted to the hospital in 2005 had high paraprotein concentration (85.75 g/l) and prolonged trombin time (76.8 s), activated partial thromboplastin time (39.5 s), prothrombin time (23.5 s) and reptilase time (72.0 s). The fibrinogen level was within normal range. The fibrin polymerization induced by both thrombin and reptilase was impaired. Scanning electron microscopy revealed abnormal clot morphology. After six months of treatment with prednison and melphalan, the paraprotein level decreased (19.48 g/l) and coagulation normalized as well as fibrin polymerization and fibrin network morphology. Conclusion: Acquired dysfibrinogenemia associated with multiple myeloma with high paraprotein level was diagnosed in the 72-yr-old patient. Acknowledgement: This work was supported by a grant of The Ministry of Health Czech Republic number 2373601. P048 The significance of ultrasonic investigation in the urgent diagnostics of internal hemorrhage in patients with hemophilia R.V. Avagyan *. Hematological Center MPH, Yerevan, Armenia During recent years the ultrasonic investigation has become a standard method in the diagnostics of internal hemorrhage in the case of blood diseases. Aim: The revelation of the significance of ultrasonic investigation method in patients with hemophilia in the case of hematoma and internal hemorrhage. Materials and Methods: 20 patients with hemophilia were included in the investigation carried out in the Hematological Center of Ministry of Public Health of Republic of Armenia. The ultrasonic examination with the help of apparatus “Melison” 128 B-W-prim: After being admitted into the clinic the patients underwent an immediate echo-examination, and free liquid and a formation in the abdominal cavity were discovered (internal hemorrhage, hematoma). By means of clinical investigation was discovered anemia and ESR increase. The method of ultrasonic investigation is a method of urgent diagnostics of internal hemorrhage for people suffering from hemophilia. P049 Could heparin-induced thrombocytopenia and thrombosis be a rare manifestation of antiphospholipid antibody syndrome? Z. Olah1 *, A. Schlammadinger1 , J. Mikita1 , P. Ilonczai1 , K. Razso1 , Z. Bereczky2 , Z. Boda1 . 1 University of Debrecen, Medical and Health Science Center, 2nd Department of Medicine, Debrecen, Hungary, 2 Clinical Research Center, Debrecen, Hungary Introduction: The pathogenesis and clinical manifestation of heparin-induced thrombocytopenia (HIT) and antiphospholipid syndrome (APS) have been shown to be remarkably similar. Additionally, increased prevalence of heparin-platelet factor4-induced antibodies was described in APS. However, presence of antiphospholipid antibodies and APS has up to now not been investigated in HITT (HIT and thrombosis). Therefore, our aim was to examine whether antiphospholipid antibodies and APS could be detected in HITT. Method: Four consecutive patients with severe HITT characterised by widespread arterial and/or venous thrombosis were examined. In all four patients, lupus anticoagulant, IgG, IgM and IgA type anti-B2GPI and anti-cardiolipin antibodies were assessed in the acute phase of HITT and 3 months later, in the follow-up period.
2 September 2007
S101
Methods: The cause of abnormal coagulation results was determined using fibrin polymerisation measurement, fibrinogen clottability measurement, sodium dodecyl sulphate polyacrylamide gel electrophoresis (SDS-PAGE), Western blotting, SEM, DNA sequencing, MALDI-TOF MS and ELISA. Results: The patient admitted to the hospital in 2005 had high paraprotein concentration (85.75 g/l) and prolonged trombin time (76.8 s), activated partial thromboplastin time (39.5 s), prothrombin time (23.5 s) and reptilase time (72.0 s). The fibrinogen level was within normal range. The fibrin polymerization induced by both thrombin and reptilase was impaired. Scanning electron microscopy revealed abnormal clot morphology. After six months of treatment with prednison and melphalan, the paraprotein level decreased (19.48 g/l) and coagulation normalized as well as fibrin polymerization and fibrin network morphology. Conclusion: Acquired dysfibrinogenemia associated with multiple myeloma with high paraprotein level was diagnosed in the 72-yr-old patient. Acknowledgement: This work was supported by a grant of The Ministry of Health Czech Republic number 2373601. P048 The significance of ultrasonic investigation in the urgent diagnostics of internal hemorrhage in patients with hemophilia R.V. Avagyan *. Hematological Center MPH, Yerevan, Armenia During recent years the ultrasonic investigation has become a standard method in the diagnostics of internal hemorrhage in the case of blood diseases. Aim: The revelation of the significance of ultrasonic investigation method in patients with hemophilia in the case of hematoma and internal hemorrhage. Materials and Methods: 20 patients with hemophilia were included in the investigation carried out in the Hematological Center of Ministry of Public Health of Republic of Armenia. The ultrasonic examination with the help of apparatus “Melison” 128 B-W-prim: After being admitted into the clinic the patients underwent an immediate echo-examination, and free liquid and a formation in the abdominal cavity were discovered (internal hemorrhage, hematoma). By means of clinical investigation was discovered anemia and ESR increase. The method of ultrasonic investigation is a method of urgent diagnostics of internal hemorrhage for people suffering from hemophilia. P049 Could heparin-induced thrombocytopenia and thrombosis be a rare manifestation of antiphospholipid antibody syndrome? Z. Olah1 *, A. Schlammadinger1 , J. Mikita1 , P. Ilonczai1 , K. Razso1 , Z. Bereczky2 , Z. Boda1 . 1 University of Debrecen, Medical and Health Science Center, 2nd Department of Medicine, Debrecen, Hungary, 2 Clinical Research Center, Debrecen, Hungary Introduction: The pathogenesis and clinical manifestation of heparin-induced thrombocytopenia (HIT) and antiphospholipid syndrome (APS) have been shown to be remarkably similar. Additionally, increased prevalence of heparin-platelet factor4-induced antibodies was described in APS. However, presence of antiphospholipid antibodies and APS has up to now not been investigated in HITT (HIT and thrombosis). Therefore, our aim was to examine whether antiphospholipid antibodies and APS could be detected in HITT. Method: Four consecutive patients with severe HITT characterised by widespread arterial and/or venous thrombosis were examined. In all four patients, lupus anticoagulant, IgG, IgM and IgA type anti-B2GPI and anti-cardiolipin antibodies were assessed in the acute phase of HITT and 3 months later, in the follow-up period.