Microsoft powerpoint - poster hemofilia adq isth 2009

TREATMENT OF ACQUIRED HEMOPHILIA A WITH RECOMBINANT ACTIVATED FVII
M. A. Cermelj, S. Ouviña, A. Guzmán, O. Torres, A. Ferro Centro de Hematología, Trombosis y Análisis Clínicos, CEHTAC, Buenos Aires, Argentina Introduction
Acquired hemophilia A ( acq He A) is a rare condition, and is due Case 1: 7 years old girl with acq He A detected during nephrotic
to the production of autoantibodies in adult life which inactivate FVIII. Typical clinical manifestations of de acquired form are corticosteroids. She presented post-traumatic subscapular haematoma and extensive cutaneous purpura and internal hemorrhage: bleeding multiple ecchymoses. APTT: 138 sec, FVIII < 1%, FVIII inhibitor titre (INH): into the joints is not a prominent feature . Both sexes are affected 59 BU/ml. She was treated with prednisone 2 mg/kg/day orally for 45 days, and there may be identifiable underlying conditions. Diagnosis is but 20 days later she presented post-traumatic retroperitoneal haematoma based on the finding of a low FVIII level associated with the and was treated with 1 g/kg gamma-globulin and corticosteroids during 30 presence of a time-dependent inhibitor in the plasma patient.
days (INH at the end of the treatment: 1793 BU/ml). After that she Treatment of the condition involves the use of an activated presented a bleeding wound in her face, and was treated with activated prothrombin complex concentrate or recombinant activated FVII prothrombin complex concentrates (INH: 1350 BU/ml) with non adequate to control bleeding episodes. In addition, immunosuppression with clinical response. She was treated with prednisone 1 mg/kg/day during 45 steroids is usually effective at reducing inhibitor production and days (INH: 2623 BU/ml). Response test to rFVIIa 90 μg/kg was performed bringing about a sustained rise in the FVIII level. We report two and INH decreased to 107 BU/ml 60 min post infusion. Episodic treatment patients with acq He A with high FVIII inhibitor titre.
with rFVIIa was selected. She only presented minor episodes of bleeding atthe moment.
Material and Methods
Case 2: 68 years old female with low grade stage III lymphoma,
PT measure was performed with Thromborel S, Dade Behring, in previously treated with CHOP schedule, 8 months later she showed a BCT coagulometer, Dade Behring, Marburg, Germany. APTT was performed with Pathromtin SL, Dade Behring, in a BCT compartamental syndrome caused by a tension based haematoma on the forearm with vascular compromise. APTT:130 sec, FVIII<1%, INH: 860 concentration was also determined in the same coagulometer by BU/ml. She received rFVIIa 90 μg/kg each 3 hours during 3 days. INH: 162 coagulometric and chromogenic methods. FVIII inhibitor was determinated through both Bethesda and Chromogenic methods.
Chromogenic method : FVIII is activated by thrombin, the resultingFVIIIa increases the conversion of FX to FXa in the presence of Conclusion:
In both patients rFVIIa provided a safe and effective treatment for bleeding determinated through the hydrolysis of FXa specific sustrate p- episodes, an adequated haemostatic cover and reduced the FVIII inhibitor nitroanilide. The realesed amount of p-nitroanilide measure to 405 nm is proportional to the activity of FXa and then to the FVIIIconcentration in the sample. The patient plasma dilution thatleaves a residual of FVIII activity of 50% has 1 unit of inhibitor ofchromogenic sustrate/ml (CS/ml).

Source: http://solucion-pymes.com.ar/!repo_monicacermelj/File/Poster%20Hemofilia%20Adq%20ISTH%202009.pdf