Response of hemophilia A with bleeding to fresh dry plasma.

Fresh dry plasma (FDP) is a lyophilized form of fresh frozen plasma (FFP) which can be stored at 4 degrees C for one year. One bottle of FDP is prepared from 220 ml of FFP and contains FVIII: C 0.75 +/- 0.3 U/ml (mean +/- 1 SD). This study describes the clinical and laboratory response in 7 severe and 4 moderate hemophilia A patients. The age ranged from 7-17 years (mean +/- SD = 11.7 +/- 2.9 years). Either 12.4 ml/kg. FDP or 12.2 ml/kg FFP was transfused to the patients when they had bleeding episodes such as hemarthrosis, hematoma. 16 episodes of FDP and 12 episodes of FFP transfusion were studied. The bleeding could be effectively controlled by FDP or FFP except one patient who had physical therapy at 8 hours post FDP transfusion. The increment of FVIII:C was 14.1 +/- 5.3% at 30 minutes after FDP transfusion and 12.1 +/- 3.7% at 30 minutes after FFP transfusion. The recovery rate was 83.2 +/- 32.6% in FDP and 65.3 +/- 22.7% in FFP transfusion. The FVIII:C was decreased to 78.9 +/- 12.3%, 55.6 +/- 13% and 16.3% of the initial level at 2, 8, 24 hours after FDP transfusion respectively which were not statistically significantly different from FFP transfusion. No serious complication was found. FDP will replace FFP for the treatment of coagulation disorders such as hemophilia A. It is an useful alternative therapy which can be provided to the hemophiliac patients in the rural area in developing countries.

High transfusion in children with beta-thalassemia/Hb E: clinical and laboratory assessment of 18 cases.

Between 1990-1992, 18 patients with beta-thalassemia/Hb E age between 2-13 years (mean 7.4 +/- 3 years) were examined. Three patients were splenectomized and the rest were nonsplenectomized. They were divided into 3 groups. Group A:5 nonsplenectomized and 3 splenectomized patients had high transfusion rates with subcutaneous desferrioxamine injections. Five patients in group B received only high transfusion whereas in 5 patients in group C the levels pretransfusion Hb were maintained between 6-7 g/dl. The mean blood consumption in the nonsplenectomized groups were 220 +/- 25.3, 221 +/- 59 and 175.4 +/- 45.4 ml/kg/year in groups A, B and C, respectively. In group A, the mean blood requirement was 40% higher in the nonsplenectomized group. In the high transfusion regimen the spleen size did not increase and serum aspartate aminotransferase showed a striking fall in the majority of cases. The absolute increases in serum ferritin were 843.2 +/- 395, 861 +/- 252 and 1,262 +/- 440 ng/ml in groups A, B and C, respectively. These data demonstrated that high transfusion with desferrioxamine injection could improve the clinical well being of the patients.