N R Selik | PubMed Health

PubMed1988

"Sticky platelet syndrome": a congenital platelet abnormality predisposing to thrombosis?

We have identified a number of patients with coronary artery disease, TIAs and/or strokes and idiopathic ischemic optic neuropathy who have a platelet population which is in vitro hyperaggregable with epinephrine and ADP and hyperresponsive to surface contact. These patients have no identifiable risk factors. Several families have been identified in which multiple members had these findings. Many, but not all have had clinical symptoms. An autosomal (dominant) pattern of heredity seems to evolve. We refer to this as "sticky platelet syndrome" and hypothesize that it may represent a congenital platelet abnormality which potentially predisposes to thromboembolisms. The precise nature of the defect is not known at this time.

Adolescent↗

PubMed1985

Combined factor V/VIII deficiency: a case report including levels of factor V and factor VIII coagulant and antigen as well as protein C inhibitor.

Comprehensive coagulation studies were performed on members of a family with combined factor V/VIII deficiency. The purpose of these studies was to investigate the hypothesis that combined factor V/VIII deficiency is due to a lack of the inhibitor to activated protein C. The analyses performed included routine APTT and PT, factor V and VIII coagulant activity and antigen levels, von Willebrand factor levels, protein C antigen assay, and both protein C inhibitor activity and antigen levels. Three of the 19 family members studied were found to have a deficiency of both factors V and VIII. These three individuals showed prolonged APTTs and PTs and decreased levels of factor V and factor VIII coagulant activity and antigen. Factor VIII related antigen and ristocetin cofactor (von Willebrand factor) levels were normal. Protein C and both protein C inhibitor activity and antigen levels were also found to be normal. These findings confirm the results of other recent investigators and indicate that the autosomal, inherited combined factor V/VIII deficiency is not due to a protein C inhibitor deficiency. The real defect in this combined deficiency remains to be determined.

Adult↗

PubMed1985

Hemostasis changes during cardiopulmonary bypass surgery.

A number of hemostasis parameters were studied in a total of 63 patients undergoing cardiopulmonary bypass (CPB) for open heart surgery. In 33 patients fibrinogen, Factors II, V, VIII:C, X, XI, antithrombin, plasminogen, alpha 2-antiplasmin, and platelet counts were assayed before surgery, during maximal hypothermia, at the end of the bypass procedure, before and after protamine sulfate infusion, in the intensive care unit, and 48 hours postoperatively. All factors assayed decreased markedly when the patients were placed on the bypass machine, the drop fairly well paralleling the decrease in hematocrit. During bypass the factors remained low, although a slight tendency toward an increase was noted. Only platelet counts remained low with a decreasing trend until the end of bypass. In the intensive care unit a second decrease in fibrinogen, Factors II and V and antithrombin was noted. This drop was unrelated to four patients who experienced a greater blood loss during this time than the others. Forty-eight hours postoperatively, a marked increase could be found in all clotting factors and near normal levels were measured. Platelet counts remained low, however. The decrease in factors rarely dropped into a range where one would expect a compromised hemostasis (less than 30%). Although antithrombin levels decreased below 60%, no difficulties with heparinization were encountered. Several factors were assayed manually and by automated analyzer (Multistat III), and excellent correlations were found between both procedures. Also a good correlation was found between the activated whole blood clotting times and quantitative heparin assays. In 30 additional patients platelet function was studied before surgery, after thoracotomy, after heparin administration, after initiation of bypass, at maximal hypothermia, before and after protamine sulfate infusion, and 24 hours postoperatively. Platelet counts once again decreased as patients were placed on the CPB machine and remained low throughout the procedure. Mean platelet volumes were unchanged until protamine was given. At that time, a significant drop in mean platelet volume was recorded. Twenty-four hours postoperatively the volumes were normal again. Platelet aggregation studies were performed on a whole blood aggregometer using two concentrations of ADP, collagen, and ristocetin as aggregation inducers. A significant decrease in aggregability was seen when the patients were connected to the CPB apparatus.(ABSTRACT TRUNCATED AT 400 WORDS)

Adult↗