The erythrocytes in paroxysmal nocturnal haemoglobinuria of intermediate sensitivity to complement lysis

Wendell F. Rosse, Sharon Hoffman, Milton Campbell, Michael Borowitz, Joseph O. Moore, Charles J. Parker

Research output: Contribution to journalArticle

Abstract

The sensitivity to lysis by complement of the erythrocytes of 56 patients with paroxysmal nocturnal haemoglobinuria (PNH) was compared to the membrane expression of decay accelerating factor (DAF, CD55), membrane inhibitor of reactive lysis (MIRL, CD59) and acetylcholinesterase (AChE). Most patients (36/50 72% in whom the analysis could be made) appeared to have erythrocytes of intermediate sensitivity to complement in the blood. These cells appeared as a discrete population of cells (PNH II cells), as a ‘tail’ of cells slightly less sensitive than the predominant PNH III cells (previously called PNH IIIb cells), or as a continuous spectrum of cells sensitive to complement. The PNH III cells totally lacked all three proteins (DAF, MIRL, AChE) by flow cytometric analysis whereas PNH I cells appeared to have normal or nearly normal amounts of each. The cells of intermediate sensitivity (PNH II) had coordinately decreased expression of all three proteins; the level of expression of DAF and MIRL paralleled the sensitivity of the cells to the haemolytic action of complement.

Original languageEnglish (US)
Pages (from-to)99-107
Number of pages9
JournalBritish journal of haematology
Volume79
Issue number1
DOIs
StatePublished - Sep 1991
Externally publishedYes

ASJC Scopus subject areas

  • Hematology

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