The relationship of clinical bleeding tendency and factor XI antigen (XI:Ag) in factor XI deficiency was studied in 78 members of 25 factor XI-deficient kindreds. Factor XI:Ag was measured in a competitive radioimmunoassay, using monospecific, heterologous anti-factor XI antibody. 125I-labeled factor XI, and staphylococcal protein A as the precipitating agent. Deficiency of factor XI clotting activity (XI:C), less than 0.62 U/mL, occurred in 48 individuals, 22 of whom experienced postoperative or posttraumatic bleeding: Their mean factor XI:C was 0.21 +/- 0.04 U/mL (SEM), and factor XI:Ag was 0.23 +/- 0.04 U/mL. The remaining 26 had no clinical bleeding, many despite surgical challenge: Their mean factor XI:C was 0.30 +/- 0.04 U/mL, and factor XI:Ag was 0.34 +/- 0.05 U/mL. In all, 13 kindreds had between 1 and 11 members with bleeding; the other 12 had none with deficient hemostasis. Two heterozygous factor XI-deficient individuals appeared to be positive for cross-reacting material (CRM+). The slope of the regression line for factor XI:C and factor XI:Ag data points in the 78 individuals tested did not differ from control, and all points fell within 95% confidence limits derived from control. In conclusion, bleeding tendency appears to be consistent within a given kindred and is not determined exclusively by factor XI:C or factor XI:Ag levels.
During the first 5 years (1981)(1982)(1983)(1984)(1985) of the liver transplantation program in Pittsburgh, a total (preoperative, intraoperative, and postoperative) of 18,668 packed red cell units, 23,627 fresh-frozen plasma units, 20,590 platelet units, and 4241 cryoprecipitate units was transfused for the procedures. This represents 3 to 9 percent of the total of blood products supplied by the Central Blood Bank to its 32 member hospitals. Six hundred thirty-six (636) transplants were performed on 485 patients in two hospitals: the Presbyterian University Hospital (564 beds) and Children's Hospital of Pittsburgh (236 beds). All of the blood components used in the operations were procured and released by the Central Blood Bank. This report describes some of these findings.The Development of liver transplantation (LTx) programs in recent years has put new demands on blood banks and transfusion services, which must devise means of coping with this new challenge. The Central Blood Bank of Pittsburgh (CBB) provides blood product support for 32 member hospitals. Two of these hospitals, Presbyterian University Hospital (PUH, 564 beds) and Children's Hospital of Pittsburgh (CHP, 236 beds) began doing liver transplants in 1981. The results of this experiment are reported here. Study ResultsFigure 1 divides the numbers of operations done in Pittsburgh by 1) the year of transplantation, 2) the hospital, and 3) the number of transplantations per patient. The numbers increased almost exponentially. Table 1 shows that, during the first 5 years, 290 adults and 195 children underwent 626 LTx. Of these, 61 adults and 54 children received two livers, and 15 adults and 11 children received three livers. Table 2 shows the components used preoperatively, intraoperatively, and postoperatively. Most packed red cells (RBCs) (64%) and cryoprecipitate (86%) were used intraoperatively. Slightly more than one-half of the fresh-frozen plasma (FFP) and less than one-half of the platelets were used in the operating room. Table 2 also compares the total number of components used for © J. B. Lippincott Co.
The spontaneous disappearance of the inhibitor to factor VIII (FVIII) was observed in two human immunodeficiency virus (HIV)-infected men with hemophilia A. Both men had end-stage HIV infection, one with acquired immune deficiency syndrome (AIDS) and one with severe AIDS-related complex (ARC). Loss of the inhibitor was associated with a fall in T4 helper lymphocytes to less than 100 per mm3 in both patients. Subsequent spontaneous and traumatic hemorrhages were treated successfully with standard doses of FVIII concentrate, resulting in adequate FVIII:C levels and good hemostasis. The mechanism by which the anti-FVIII inhibitor disappears is not known, but it is likely to be related to a quantitative decline in T4 cell number.
A 10-year-old boy had a severe lifelong hemorrhagic disorder that had necessitated more than 50 hospitalizations. Laboratory examination showed prolonged bleeding, clotting, partial thromboplastin, prothrombin, and thrombin times. These findings were due to a potent inhibitor of the thrombin-fibrinogen reaction. This inhibitor was similar to heparin in that it acted immediately and did not interfere with the coagulant activities of certain venoms. It differed from heparin in not being adsorbed to barium citrate or neutralized by protamine sulfate. The inhibitory effect was found in the alpha1-globulin fraction. It was identified immunologically and functionally as a double-banded alpha1-antitrypsin of a previously unreported phenotype. The inhibitory effects were depressed by trypsin and heterologous anti-alpha1-antitrypsin.
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