Paroxysmal Nocturnal Hemoglobinuria

  • W. F. Rosse
Part of the Current Topics in Microbiology and Immunology book series (CT MICROBIOLOGY, volume 178)


Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal disorder of the hematopoietic stem cell (Hartmann and Arnold 1977) resulting in the production of blood cells which are defective in that they lack or are markedly deficient in glycan-phosphatidylinositol (GPI)-linked surface proteins (Rosse 1990a). To date, 11 such proteins have been found to be missing or deficient on the abnormal cells (Auditore et al. 1960; Kunstling and Rosse 1969; Beck and Valentine 1951; Burroughs et al. 1988; Nicholson-Weller et al. 1983a; Pangburn et al. 1983; Holguin et al. 1989a; Hänsch et al. 1987; Selvaraj et al. 1987,1988; Simmons et al. 1989; van der Schoot et al. 1989) (see Table 1). Other GPI-linked proteins that have not been identified are probably also missing.


Paroxysmal Nocturnal Hemoglobinuria Complement Regulatory Protein Decay Accelerate Factor Paroxysmal Nocturnal Hemoglobinuria Clone Paroxysmal Nocturnal Hemoglobinuria Patient 
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Copyright information

© Springer-Verlag Berlin Heidelberg 1992

Authors and Affiliations

  • W. F. Rosse
    • 1
  1. 1.Division of Hematology/OncologyDuke University School of MedicineDurhamUSA

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