Effect on Blood Coagulation of Massive Intravascular Haemolysis

Abstract

This study was carried out on twenty-eight Sardinian subjects undergoing massive intravascular hemolysis after ingestion or inhalation of fava beans. The patients were all deficient in glucose-6-phosphate dehydrogenase but otherwise hematologically normal when investigated after the acute hemolytic episode. Prothrombin time, thrombin time and activated partial thromboplastin time were found to be shortened in the days following the hemolytic crisis. Marked increase of factor VIII (antihemophilic factor) and fibrinogen was also observed, together with a less pronounced rise of factor V. The other clotting factors were within the normal range, and fibrinogen degradation products were not present in serum. The observed rise was found to be generally proportional to the degree of red cell destruction. Progressive normalization of the abnormal parameters followed the recovery from acute hemolysis. These observations are discussed in relation to previous findings and to the occurrence of intravascular coagulation following massive red cell destruction.

Coagulation Changes during the Steady State in Homozygous Sickle‐Cell Disease in Jamaica

“…Thc cause of the elevated factor VIII is unknown. Increases in factor VIII occur in a wide variety of physiological and pathological conditions (Librc et al, 1968) including hacmolytic anaemia (Mannucci et al, 1969;Ricdlcr et al, 1968). However, changes in factor VIII levels alone are not sufkicntly specific to diagnose a hypercoagulable state.…”

“…In haeniolytic anaemia associated with cardiac valve prostheses, the mechanical damage is independent of coagulation activation as assessed by FDP levels (Slater et al, 1973) and in favism (Mannucci et al, 1969) the selective effect on the red cells does not cause elevation of FDP, although FDP measurements in this study were performed using an insensitive technique. In transfusion rcactions (Sack & Nefa, 1970), and drowning (Riedler et al, 1968), there is transient intravascular coagulation with elevation of FDP levels followed by prolonged intravascular fibrinolytic activation.…”

Disseminated Intravascular Coagulation: The Application and Utility of Diagnostic Tests

“…51,66 This is probably antigen-antibody mediated, since glucose-6phosphate dehydrogenase associated hemolysis is not associated with DIC. 106 The two final common pathways in the activation of DIC in man appear to be tissue thromboplastin release and endothelial damage with subsequent activation of complex events that may potentially initiate the intrinsic pathway. Unfortunately, it is not currently possible to quantitate the relative biologic importance of the various individual mechanisms involved in intrinsic pathway activation, and the presence of tissue thromboplastin in the human circulation has yet to be demonstrated in the appropriate clinical setting.…”

Linking Labile Heme with Thrombosis

“…As such, thrombotic events contribute to a variety of clinical sequelae encompassing ischemic heart disease and stroke [ 2 , 6 , 11 ]. Moreover, several hemolytic conditions, such as sickle cell disease (SCD) [ 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 ], hemolytic uremic syndrome [ 25 , 26 ], hemoglobinuria [ 27 , 28 ], hemolytic transfusion reactions [ 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 ], hemorrhage [ 33 , 37 , 38 , 39 , 40 , 41 , 42 ], and cardiac surgery [ 43 , 44 ], were reported to manifest thrombotic complications as thrombosis, hypercoagulability, and vasculopathy [ 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 ]. In adult SCD patients, venous thromboembolism (VTE) is even one of the leading causes of death [ 57 , 58 ].…”