The Action of Two Sulfhydryl Compounds on Normal Human Red Cells

• Published in: Blood Vol. 25; no. 4; pp. 502 - 510
• Main Authors: Sirchia, Girolamo, FERRONE, SOLDANO, Mercuriali, Francesco
• Format: Journal Article
• Language: English
• Published: Elsevier Inc 01.04.1965
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood.V25.4.502.502

PAROXYSMAL NOCTURNAL HEMOGLOBINURIA (PNH) is an uncommon hemolytic disorder caused by an intrinsic defect of the erythrocytes about which very little is known. This may, at least in part, depend on the relative rarity of the disease and on the impossibility of reproducing the characteristic lesion of the erythrocytes in the laboratory. Attempts along this line were made by Rodbard1who, assuming that the erythrocytic defect was localized in the lipid fraction of the stroma, succeeded on a few occasions in obtaining lysis of normal red cells in acidified serum—which occurs also under the same conditions to PNH red cells2—by means of repeated washing with dilute alcoholic solutions. However, the results were not constant and reproducible. By contrast, the results obtained by Metz et al.,3 using an acetylcholinesterase inhibitor ( OMPA ), and by Ghiotto et al. ( quoted by De Sandre et al.4 ) using another acetylcholinesterase inhibitor (physostigmine), were complete failures. So far, the best results have been obtained by Yachnin et al.,5 who have reported that, when normal red cells are altered by means of various proteolytic enzymes ( trypsin, ficin, bromelin and papain ), cholera vibrio filtrate, influenza virus or sodium periodate, it is possible to obtain erythrocytes whose in vitro behaviour is similar to that of PNH red cells, although differing from the latter in some respects. The purpose of this report is to describe the results obtained by treating normal human red cells with AET ( 2-amino-ethylisothiouronium bromide ) and with cysteine; both substances contain SH groups, belong to the group of sulfhydryl compounds, but are not enzymes in nature.