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Paroxysmal Nocturnal Hemoglobinuria

  • Chapter
Book cover Membrane Defenses Against Attack by Complement and Perforins

Part of the book series: Current Topics in Microbiology and Immunology ((CT MICROBIOLOGY,volume 178))

Abstract

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.

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Authors and Affiliations

  1. Division of Hematology/Oncology, Duke University School of Medicine, Durham, NC, 27710, USA

    W. F. Rosse

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  1. W. F. Rosse
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  1. Department of Veterans Affairs Medical Center, University of Utah School of Medicine, Salt Lake City, Utah, 84148, USA

    Charles J. Parker A. B., M.D. (Associate Professor of Medicine, Chief, Hematology Subsection) (Associate Professor of Medicine, Chief, Hematology Subsection)

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Rosse, W.F. (1992). Paroxysmal Nocturnal Hemoglobinuria. In: Parker, C.J. (eds) Membrane Defenses Against Attack by Complement and Perforins. Current Topics in Microbiology and Immunology, vol 178. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-77014-2_10

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  • DOI: https://doi.org/10.1007/978-3-642-77014-2_10

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