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Studies of Paroxysmal Nocturnal Hemoglobinuria Erythrocytes: Increased Lysis and Lipid Peroxide Formation by Hydrogen Peroxide

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Studies of Paroxysmal Nocturnal Hemoglobinuria Erythrocytes: Increased Lysis and Lipid Peroxide Formation by Hydrogen Peroxide Studies of Paroxysmal Nocturnal Hemoglobinuria Erythrocytes: Increased Lysis and Lipid Peroxide Formation by Hydrogen Peroxide Charles E. Mengel, … , Herbert E. Kann Jr., Wilhelm D. Meriwether J Clin Invest. 1967;46(11):1715-1723. https://doi.org/10.1172/JCI105662. Research Article When paroxysmal nocturnal hemoglobinuria (PNH) erythrocytes were exposed to H2O2 they lysed excessively and formed greater than normal quantities of lipid peroxides when compared to red cells of normal subjects and patients with most types of hematologic disease. It was also shown that lytic sensitivity to acidified serum was related to the enhanced lytic sensitivity to H2O2. If the lipid of PNH cells was first extracted then exposed to ultraviolet radiation more lipid peroxides were formed than in extracts of normal red blood cells. The possible explanations for these findings and their relationship to the PNH hemolytic mechanism are discussed. Find the latest version: https://jci.me/105662/pdf The Journal of Clinical Investigation Vol. 46, No. 11, 1967 Studies of Paroxysmal Nocturnal Hemoglobinuria Erythro- cytes: Increased Lysis and Lipid Peroxide Formation by Hydrogen Peroxide * CHARLES E. MENGELt HERBERT E. KANN, JR.,§ AND WILHELM D. MERIWETHER (From the Department of Medicine, The Ohio State University, College of Medicine, Columbus, Ohio) Abstract. When paroxysmal nocturnal hemoglobinuria (PNH) erythro- cytes were exposed to H202 they lysed excessively and formed greater than normal quantities of lipid peroxides when compared to red cells of normal subjects and patients with most types of hematologic disease. It was also shown that lytic sensitivity to acidified serum was related to the enhanced lytic sensitivity to H202. If the lipid of PNH cells was first extracted then ex- posed to ultraviolet radiation more lipid peroxides were formed than in ex- tracts of normal red blood cells. The possible explanations for these findings and their relationship to the PNH hemolytic mechanism are discussed. Introduction We have previously described greatly enhanced Paroxysmal nocturnal hemoglobinuria (PNH) in vivo PNH hemolytic activity after parenteral is a unique hemolytic anemia characterized by ac- iron administration in several patients with PNH celerated intravascular hemolysis during sleep (12). This was presumed to reflect a unique (1). Although the disease is usually considered effect of iron on PNH erythrocytes. Iron serves to be acquired, an abnormality intrinsic to the red as a catalyst in the peroxidation of lipid (13, 14), cell is responsible for hemolysis (2-6). Exten- and increased levels of lipid peroxides have been sive studies have helped to define the role of com- found in tissues of animals injected with iron com- plement components in lysis of PNH red cells plexes (15). Recent studies in our laboratory (7-11), but the nature of the intracorpuscular de- have shown that peroxidation of red cell lipid can fect has not been clarified. occur in vivo causing damage and lysis of red cells (16). These observations led us to speculate * Received for publication 10 November 1966 and in re- vised form 10 July 1967. that PNH red cells might be unusually sensitive to Presented in part before the Central Society for Clini- lipid prooxidants, and that lipid peroxidation might cal Research, November 1965, Chicago, Ill. be involved in PNH hemolysis. Supported by U. S. Public Health Service Research In the course of studies undertaken to investi- Grants CA-08699 and CA-08702, and by U. S. N. Re- gate this possibility, it was found that search Contract Nonr-495 (30), and U. S. Public Health (a) PNH Service Training Grant CA-5192-01. red cells are abnormally susceptible to lysis by t Markle Scholar in Academic Medicine, Director, HO,, an agent capable of peroxidizing lipid, and Division of Hematology and Oncology, The Ohio State (b) under the experimental conditions cited University, Columbus, Ohio. peroxidation was induced more rapidly in lipid §Research Fellow, Division of Hematology and On- of PNH cology, The Ohio State University, Columbus, Ohio. red cells than in lipid of normal red 11 Research Trainee, Division of Hematology and On- cells. cology, The Ohio State University University Hospitals, Columbus, Ohio, and Medical Student, Duke University Methods School of Medicine, Durham, N. C. Patients Address requests for reprints to Dr. Charles E. Mengel, Division of Hematology and Oncology, The Department Blood samples from 14 patients with PNH, several of of Medicine, The Ohio State University, College of whom have been described in detail previously (12, 17), Medicine, Columbus, Ohio 43210. were studied and compared to blood from a control 1715 1716 MENGEL, KANN, AND MERIWETHER group consisting of 300 normal subjects and 100 patients equal volumes of H202 solutions in buffer at pH 7.4, in- with many different hematologic diseases. cubated at 37°C for 15 min and then at room tempera- Red cells from all PNH patients consistently lysed in ture for 2 hr and 45 min. Buffer blanks were carried acidified serum; lysis was completely prevented by heat- throughout. When incubation was complete 9 volumes of ing serum at 560C for i hr. Red cell acetylcholinesterase saline were added to three tubes of each H202 concentra- activity was definitely lower than normal in ten, "low tion and 9 volumes of water to the fourth. After centri- normal" in two, and within the normal range in two fugation the supernatant was read at 540 m/u after con- others. All but 2 patients (No. 4 and No. 7) had version of hemoglobin to cyanmethemoglobin. moderately severe hemolytic activity during the period (e) Lipid peroxide determinations. Lipid peroxide of follow-up and study. Patients included in the con- formation in erythrocytes was determined by measuring trol group had most types of hematologic diseases in- the pink chromagen (absorbance maximum 535 mt) cluding various types of erythrocyte glucose-6-phosphate formed by the reaction of 2-thiobarbituric acid (TBA) dehydrogenase (G-6-PD) deficiency, megaloblastic ane- with a breakdown product of lipid peroxides, malonyl- mias, hemoglobinopathies, immune hemolytic anemia, aldehyde (22, 23). Erythrocytes were washed twice in aplastic and refractory anemias, and various myelo- and physiologic saline. Then 0.05 ml of red cells in 1 ml of lymphoproliferative disorders. physiologic saline was mixed with 1 ml of the 6% H202 solution and incubated for 1 hr at 37°C. 1 ml of this Studies of erythrocytes incubation mixture was then added to 1 ml of 10% tri- (a) Hemoglobin was determined as cyanmethemoglobin, choloroacetic acid and after thorough mixing was filtered hematocrits by a micro-method, and reticulocyte counts through Whatman No. 1 paper. 1 ml of the filtrate was and Heinz body preparations by standard methods (8). mixed well with 1.2 ml of thiobarbituric acid (Eastman (b) Fresh serum, kept under oil at pH 6.2 + 0.1, was Organic Chemicals, Rochester, N. Y.) (0.67% in water) used for testing acid-serum lysis (18). and the mixture was heated in a boiling water bath for (c) Erythrocyte acetylcholinesterase activity was de- 15 min. After cooling to room temperature, absorption termined using the method described by Ellman et al. spectra were taken and the absorbance at 535 mu was (19), except that a change of absorbance of 0.001 was recorded. taken as one enzyme unit, and values were expressed as A standard absorption curve for malonylaldehyde was enzyme units per microliter cells per minute. prepared using 1, 1, 3, 3-tetraethoxypropane (TEP) (d) Hydrogen peroxide hemolysis test. Details of (Kay-Fries Chemicals, Inc., New York, N. Y.), a com- the method used for determining in vitro lytic sensitivity pound that hydrolyzes to 1 mole of malonylaldehyde and of erythrocytes to hydrogen peroxide have been described 4 moles of ethanol (24). An absorbance of 0.1 in the previously (16, 20, 21). All glassware used was washed TBA reaction was calculated to be equivalent to 8 with concentrated nitric acid and hydrogen peroxide m,umoles of malonylaldehyde. The results of all lipid (1000 ml of acid plus 5 ml of 30% H202 and thoroughly peroxide determinations were then expressed as mjumoles rinsed with thrice deionized water. Hydrogen peroxide of malonylaldehyde per milliliter of RBCs. We have pre- solutions (Becco Chemical Div., FMC Corp., Buffalo, viously discussed the advantages and limitations of the N. Y.), 2-6%, prepared in buffer (0.2 M KH2PO4-0.2 TBA test for lipid peroxides (16). M NaOH) at pH 7.4, were stored away from light at (f) Whole blood methemoglobin levels were deter- 4°C. Each H202 solution was titrated iodometrically for mined spectrophotometrically (25). G-6-PD activity was actual peroxide content and readjusted to the desired determined as described by Zinkham et al. (26), except concentration when necessary. Erythrocytes from de- that a change of absorbance of 0.001 was taken as one fibrinated blood were made up to 2.5% suspensions (cor- enzyme unit and activities were expressed as enzyme units rected for the hematocrit of packed cells) in a solution per microliter of cells per minute. Erythrocyte catalase of equal volumes of buffer (250 ml of 0.2 M KH2PO4 and activity was determined as described by Feinstein (27). 197 ml of 0.2 M NaOH diluted to 1000 ml at pH 7.4), and Red cell glutathione peroxidase activity was determined physiologic saline and were incubated for 1 hr at 37°C. by a method described previously (16) and blood re- Cells were then washed once and made up in 5% suspen- duced glutathione was measured as described by Beutler sions in physiologic saline. These suspensions checked et al. (28). by hemoglobin and red blood cell (RBC) counts were reproducible in triplicate samples of each subject and Studies of red cell lipid were identical for the control subject and PNH patients Lipid extractions were performed simultaneously on in each simultaneous study, as were the final 100% he- PNH and normal erythrocytes in each study.
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