Platelet, erythrocyte, and fibrinogen kinetics in the hemolytic-uremic syndrome of infancy

Hemolytic uremic syndrome–associated Shiga toxins promote endothelial-cell secretion and impair ADAMTS13 cleavage of unusually large von Willebrand factor multimers

“…1 In one study of 4 infants with HUS examined 3 to 6 days after the appearance of symptoms, platelet lifespan was shortened in all patients. 52 Thrombocytopenia, glomerular and peritubular capillary platelet microthrombi (observed by electron microscopy) and fibrin, and acute renal failure have also been observed in the baboon model of Stx-induced HUS. 15,32 In one study of 4 patients who died of Stx-associated HUS, 43 fibrin (but not VWF antigen) was detected in increased quantity in the glomerular thrombi of 3 patients.…”

Shiga Toxin/Verocytotoxin-Producing Escherichia coli Infections: Practical Clinical Perspectives

“…Several additional elements should be considered when pondering the need for erythrocyte transfusion. First, erythrocyte life span is short in HUS, ranging between 8 and 24 days (101), and the fibrin debris presumably recedes on a day-by-day basis. Hence, transfused red cells might last longer if transfusion can be delayed until needed.…”

“…We also rarely transfuse platelets because the underlying process leading to thrombocytopenia is most likely entrapment of platelets in thrombi, and thrombocytes have short circulating half-lives in HUS (101). Also, HUS is a thrombotic process, which is not well served by platelet transfusions.…”

“…It is also concerning that most HUS-related strokes are thrombotic and not hemorrhagic (102,103). Fibrinogen turnover is not increased in HUS as it is in classic consumptive coagulopathies (101). We therefore recommend against requesting disseminated intravascular coagulation laboratory tests as they are not likely to provide relevant information.…”

Thrombolytic Therapy in Haemolyticuraemic Syndrome

“…Laboratory studies to detect continuing intravascular coagulation have been conflicting but have usually shown normal or high coagulation factor levels despite persistent thrombocytopenia (Gilchrist et al, 1969;Sanchez Avalos et al, 1970;Katz et ai., 1971). Isotope labelling of platelets and fibrinogen has also failed to show continuing intravascular consumption, and the results of organ scanning have suggested that the persistence of thrombocytopenia may be due to splenic sequestration rather than renal deposition of damaged platelets (Metz, 1972;Katz et al, 1973). A few cases of the syndrome studied early in the disease have, however, shown low levels of coagulation factors, suggesting continuing consumption, with a subsequent rebound to high levels (Willoughby et al, 1972).…”