The Lysis of Paroxysmal Nocturnal Haemoglobinuria Red Cells by Serum and Cobra Factor

Makaleler > Tam Makale · 1972

Yazarlar: Tomris Kabakĉi, W. F. Rosse, G. L. Logue

British Journal of Haematology, Uluslararası, 1972, cilt 23, ss 693-705

DOI: 10.1111/j.1365-2141.1972.tb03484.x

Özet

Summary. The complement‐sensitive red cells of patients with paroxysmal nocturnal haemoglobinuria (PNH) are haemolysed in the presence of serum and a protein factor present in cobra venom (CoF). The reaction of CoF and a serum factor, C3 proactivator, requires Mg2+. Once this interaction has taken place, the activation of C3 and the terminal components of complement can occur in the presence of EDTA and result in the lysis of PNH red cells. The concentration of reactants may be adjusted so that all of the complementsensitive red cells of PNH patients and the red cells of no other patients are lysed. A simple but specific and precise test for the presence of the complement‐sensitive red cells of PNH was devised. Normal human red cells treated with trypsin and neuraminidase are not lysed, whereas these cells treated with glutathione or 2‐aminoethylisothiouronium bromide (AET) are lysed.

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