Membrane proteins in paroxysmal nocturnal haemoglobinuria

Abstract

A review of recent information on the abnormalities of the blood cell membrane in paroxysmal nocturnal haemoglobinuria (PNH) is presented, with a detailed analysis of biochemical and flow cytometry findings. The complex patterns observed in the various cell lineages of which the PNH clone consists are described, and a simplified monoclonal antibody panel is defined for diagnostic purposes. Available data on in vitro culture of progenitor cells and on the recent establishment of PNH cell lines are summarized. Finally, we discuss speculative hypotheses on the growth advantage of the PNH clone.