Studies of beta-thromboglobulin, platelet factor 4, and fibrinopeptide A in erythrocytosis due to cyanotic congenital heart disease.

Plasma and serum assays of beta-thromboglobulin (BTG) and platelet factor 4 (PF4), and plasma fibrinopeptide A (FPA) were measured in adults with cyanotic congenital heart disease to characterize further the hemostatic disorder. Artifactual elevations of plasma BTG, PF4, and FPA appeared to occur occasionally when a 21 or 22 gauge needle was used to collect blood. The high packed red cell volume was probably the cause. Use of a larger caliber needle (20 gauge) appeared to lessen the problem. Normal plasma FPA levels (20 gauge needle) in 8 of 9 patients suggest that chronic intravascular coagulation is not common in these patients. Serum BTG and PF4, used to estimate total platelet content of these proteins, were normal to slightly increased. That levels were not reduced implies that platelets do not usually circulate in a "spent" state. Therapeutic phlebotomy in 8 patients was associated with small decreases in plasma BTG and PF4 of uncertain clinical significance. Five of 14 patients had elevated plasma BTG with normal to only minimally increased plasma PF4. However, 10 of 10 patients tested were found to have reduced creatinine clearance, and therefore the relative contributions of platelet secretion and reduced BTG catabolism in the kidney to elevated plasma BTG levels are unclear.