Membrane proteins in paroxysmal nocturnal haemoglobinuria

Blood Rev. 1993 Jun;7(2):75-86. doi: 10.1016/s0268-960x(05)80017-7.

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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adult
  • Antigens, CD / blood
  • Blood Cells / chemistry*
  • Cells, Cultured
  • Erythrocyte Membrane / chemistry*
  • Erythroid Precursor Cells / pathology
  • Glycosylphosphatidylinositols / blood
  • Glycosylphosphatidylinositols / deficiency*
  • Hemoglobinuria, Paroxysmal / blood*
  • Hemoglobinuria, Paroxysmal / diagnosis
  • Humans
  • Immunophenotyping
  • Membrane Proteins / blood*
  • Middle Aged
  • Models, Biological

Substances

  • Antigens, CD
  • Glycosylphosphatidylinositols
  • Membrane Proteins