Disease relevance of Hemolysis

• Hydroxyurea increases the production of fetal hemoglobin in patients with sickle cell anemia, inhibiting the polymerization of hemoglobin S and potentially improving vaso-occlusive manifestations and hemolysis [1].
• Paroxysmal nocturnal hemoglobinuria (PNH), which is characterized by intravascular hemolysis and venous thrombosis, is an acquired clonal disorder associated with a somatic mutation in a totipotent hematopoietic stem cell [2].
• A total of 126 patients (88%) were treated with ribavirin, although its use was associated with significant toxicity, including hemolysis (in 76%) and decrease in hemoglobin of 2 g/dL (in 49%) [3].
• Pyrimidine-5'-nucleotidase deficiency, an enzymopathy of nucleotide metabolism, is characterized by intracellular accumulations of pyrimidine-containing nucleotides, marked basophilic stippling on the stained blood film, splenomegaly, and hemolysis [4].
• Anemia occurs in more than one half of patients with systemic lupus erythematosus and is usually attributed to "chronic disease." Approximately 10% of patients with a positive Coombs' test manifest clinically significant hemolysis [5].

Psychiatry related information on Hemolysis

• Time-response curves revealed that erythrocyte hemolysis did not occur during the first 30 min of incubation with ionomycin, when the membrane skeleton-bound PFK was activated [6].
• The aim of this study was to determine whether genetic variability in the encoding of genes for glutathione S-transferase M1 (GSTM1) and glutathione S-transferase T1 (GSTT1) contributes to individual differences in susceptibility to pre-eclampsia, eclampsia, or hemolysis, elevated liver enzymes, and low platelets (HELLP syndrome) [7].

High impact information on Hemolysis

• Metformin-induced hemolysis with jaundice [8].
• As compared with hydroxyurea alone, treatment with hydroxyurea and erythropoietin decreased the mean (+/- SD) serum indirect bilirubin level from 0.8 +/- 0.2 to 0.5 +/- 0.1 mg per deciliter (13.3 +/- 2.9 to 8.9 +/- 2.2 mumol per liter) (P = 0.02), suggesting a further decrease in hemolysis [1].
• GM-CSF and accelerated hemolysis [9].
• Hemolysis from exposure to naphthalene mothballs [10].
• In about 20 per cent of patients taking the antihypertensive agent methyldopa, IgG autoantibodies form against red cells, but most such patients do not have hemolysis [11].

Chemical compound and disease context of Hemolysis

• In all three patients given hydroxyurea, treatment with this drug was associated with reduced hemolysis, shown by decreases in serum bilirubin and lactic dehydrogenase and prolongation of red-cell survival [12].
• Reduced chronic hemolysis during high-dose vitamin E administration in Mediterranean-type glucose-6-phosphate dehydrogenase deficiency [13].
• Since these cellular abnormalities are associated with accelerated hemolysis in vivo, the data suggest that aspirin therapy may aggravate hemolysis in patients with pyruvate kinase deficiency whose erythrocyte manifest sensitivity to salicylate in vitro [14].
• Formaldehyde-induced hemolysis during chronic hemodialysis [15].
• We conclude that hydroxyurea is effective in increasing the production of fetal hemoglobin, which in this study was found to be associated with a small decrease in hemolysis and an increase in hemoglobin levels despite myelosuppression [16].

Biological context of Hemolysis

• Homozygous human C3 deficiency. The role of C3 in antibody production, C-1s-induced vasopermeability, and cobra venom-induced passive hemolysis [17].
• Bilirubin, total calcium, biliary lipids, beta-glucuronidase activities, and cholesterol saturation indices in bile were measured, and markers of hemolysis and ineffective erythropoiesis in blood were assessed [18].
• Clones expressing 6 of 10 activities tested were identified in the library, namely, ampicillin resistance, zwittermicin A resistance, esculin hydrolysis, hemolysis, orange pigment production, and lecithinase activity [19].
• Genetic differences in hemoglobin influence on erythrocyte oxidative stress hemolysis [20].
• This enhanced complement activation may explain the increased hemolysis sometimes reported in PNH patients treated with heparin, and suggests that heparin may aggravate the consequences of pathologic alternative pathway complement activation in other diseases [21].

Anatomical context of Hemolysis

• Addition of saline-adenine-glucose solutions (40 to 100 ml per blood unit) to buffy-coat-poor red-cell concentrates allowed storage for as long as 35 days with 24-hour erythrocyte post-transfusion survival of 83 +/- 6.8 per cent (+/0 S.D.). Potassium leakage was lower, and in vitro hemolysis somewhat higher than that of whole blood [22].
• To ascertain the clinical significance of dimethyl sulfoxide-induced pigmenturia, we evaluated renal function and indicators of systemic hemolysis in stable quadriplegic patients receiving the drug intravenously (IV) for spinal cord injury [23].
• At low temperatures (on ice), PFP binds to erythrocyte membranes without producing hemolysis [24].
• The proliferation of erythroblasts after MuEV infection in animals not receiving poly I-poly C appeared to be an erythropoietin-dependent compensatory response to hemolysis [25].
• Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal hematopoietic stem cell disorder characterized by complement-mediated hemolysis due to deficiencies of glycosylphosphatidylinositol-anchored proteins (GPI-APs) in subpopulations of blood cells [26].

Gene context of Hemolysis

• These proteins include the complement inhibitors CD55 and CD59, and this explains the hypersensitivity to complement of red cells in PNH patients, manifested by intravascular hemolysis [27].
• Specifically, we found no evidence that G6PD enhanced the severity of hemolysis or increased the incidence of acute anemic episodes or sepsis in HbSS [28].
• Defective recovery and severe renal damage after acute hemolysis in hemopexin-deficient mice [29].
• Both subunits of clusterin interact with C9 and are similarly potent in inhibiting C5b-9-mediated hemolysis and Zn+(+)-induced C9 polymerization [30].
• The interference of hemolysis by BII-1 could not be explained by inhibition of activation of C4B or inhibition of C3 or C5 convertase activity [31].

Analytical, diagnostic and therapeutic context of Hemolysis

• Massive hemolysis caused by acetaminophen. Positive determination by direct Coombs test [32].
• Using a rapid, highly sensitive immunoprecipitin nephelometric technique, a retrospective study was undertaken to evaluate the clinical usefulness of determining serum haptoglobin in the diagnosis of hemolysis [33].
• Hepcidin mRNA levels were assessed in two models of anemia, acute hemolysis provoked by phenylhydrazine and bleeding provoked by repeated phlebotomies [34].
• The binding of phosphofructokinase and aldolase to the membrane of the intact human erythrocyte was assessed by the rapid hemolysis/filtration method of Kliman and Steck (Kliman, H. J., and Steck, T. L. (1980) J. Biol. Chem. 255, 6314-6321) [35].
• We describe a 45-year-old man in whom renal failure and anemia developed, without evidence of hemolysis, five months after beginning chemotherapy with mitomycin, fluorouracil, and doxorubicin hydrochloride [36].

References