Seminars in thrombosis and hemostasis
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Semin. Thromb. Hemost. · Oct 1990
ReviewNeonatal purpura fulminans due to homozygous protein C or protein S deficiencies.
Homozygous protein C deficiency or homozygous protein S deficiency are rare genetic diseases with catastrophic and fatal purpura fulminans-like or thrombotic complications occurring during the neonatal period. These diseases can now be successfully treated. Purpura fulminans is at least in part a cutaneous manifestation of the syndrome of systemic DIC. ⋯ It is very similar to the lesions seen in idiopathic purpura fulminans, warfarin-induced skin necrosis, and acute infectious purpura fulminans. Unfortunately, our current understanding of the mechanism or mechanisms of the induction and propagation of the purpura fulminans-like lesions in homozygous protein C or protein S deficiencies is minimal, since it has never been studied. We can only speculate on the mechanism based on laboratory data and comparison with the little that is known about the other similar types of lesions.
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Semin. Thromb. Hemost. · Jul 1985
ReviewHemostasis defects associated with cardiac surgery, prosthetic devices, and other extracorporeal circuits.
This discussion has provided a review of the available literature regarding alterations of hemostasis associated with CPB surgery, the use of prosthetic devices, and apheresis. The key to prevention of CPB hemorrhage is to obtain an adequate preoperative workup. Of extreme importance is an adequate history with respect to bleeding tendencies and thrombotic tendencies in both the patient and the family; of equal importance is a careful history regarding the use of drugs affecting hemostasis, especially drugs known to interfere with platelet function. ⋯ In addition to the usual prothrombin time, partial thromboplastin time, and platelet count, a standardized template bleeding time (and thrombin time in patients subjected to CPB) should be performed. The use of these simple testing modalities will guard against significant defects in vascular and platelet function. Most instances of nontechnical surgical and cardiovascular surgical hemorrhage are due to several well-defined defects in hemostasis that should be readily controlled if approached in a logical manner as a team effort among surgeons, pathologists, and hematologists.