1991
DOI: 10.1182/blood.v78.3.820.820
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Molecular basis of the enhanced susceptibility of the erythrocytes of paroxysmal nocturnal hemoglobinuria to hemolysis in acidified serum

Abstract: When incubated in acidified serum, the erythrocytes of paroxysmal nocturnal hemoglobinuria (PNH) are hemolyzed through activation of the alternative pathway of complement (APC), but normal erythrocytes are resistant to this process. PNH cells are deficient in decay- accelerating factor (DAF), a complement regulatory protein that inhibits the activity of both the classical and the alternative pathways. However, deficiency of DAF alone does not account entirely for the aberrant effects of acidified serum on PNH … Show more

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Cited by 106 publications
(37 citation statements)
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“…The cause of hypercoagulability in PNH is not clear. Increased procoagulant activity of platelets lacking CD55 and CD59, increased platelet production, and a fibrinolytic system defect due to GPI-associated u-PAR deficiency may explain the mechanism of thrombotic complications (Wilcox et al, 1991;Holguin & Parker, 1992;Ploug et al, 1992;Gralnick et al, 1995;Hugel et al, 1999).…”
Section: Discussionmentioning
confidence: 99%
See 1 more Smart Citation
“…The cause of hypercoagulability in PNH is not clear. Increased procoagulant activity of platelets lacking CD55 and CD59, increased platelet production, and a fibrinolytic system defect due to GPI-associated u-PAR deficiency may explain the mechanism of thrombotic complications (Wilcox et al, 1991;Holguin & Parker, 1992;Ploug et al, 1992;Gralnick et al, 1995;Hugel et al, 1999).…”
Section: Discussionmentioning
confidence: 99%
“…Deficiencies of CD55 (decay acceleration factor, DAF) and CD59 (membrane inhibitor of reactive lysis, MIRL) proteins result in intravascular hemolysis, whereas deficiency of urokinase plasminogen activator receptor (u-PAR) is thought to be an important factor giving rise to susceptibility to thrombosis in these patients (Wilcox et al, 1991;Holguin & Parker, 1992;Ploug et al, 1992). There are also reports indicating that increased platelet activation may contribute to thrombotic tendency (Gralnick et al, 1995;Hugel et al, 1999).…”
mentioning
confidence: 99%
“…With regard to the alternative pathway, it has been long understood that the pathway that initiates hemolysis in patients with PNH is the alternative pathway, and this apparently occurs by the tickover mechanism as hemolysis can occur spontaneously (77). Of note, hemolysis is increased at night because the slightly lower blood pH that is found during sleep accelerates the tickover mechanism.…”
Section: Pnhmentioning
confidence: 99%
“…Hemolytic anemia, thrombophylia, and cytopenia are clinical hallmarks of PNH [1][2][3][4][5]. The lack of the GPI-linked molecules CD55 (decay-accelerating factor) and CD59 (membrane inhibitor of reactive lysis), involved in the protection of red cells from the lytic attack of activated complement fractions [6], causes a chronic hemolysis with capricious exacerbations. Platelet surface activation by small amounts of complement and urokinase plasminogen activator receptor deficiency [7] might explain thrombophylia; however, the origin of the underlying bone marrow failure is still unknown [8 -10].…”
Section: Introductionmentioning
confidence: 99%