Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Editorial
. 2010 Apr;95(4):523-6.
doi: 10.3324/haematol.2009.017848.

Paroxysmal nocturnal hemoglobinuria and eculizumab

Lucio Luzzatto, Antonio Maria Risitano, Rosario Notaro
Editorial

Paroxysmal nocturnal hemoglobinuria and eculizumab

Lucio Luzzatto et al. Haematologica. 2010 Apr.
No abstract available

PubMed Disclaimer

Figures

Figure 1.
Figure 1.
The complement cascade regulation and erythrocytes. (A) Normal erythrocytes are protected from complement activation and subsequent hemolysis by CD55 and CD59. These two proteins, being GPI-linked, are missing from the surface of PNH red cells as a result of a somatic mutation of the X-linked PIG-A gene, that encodes a protein required for an early step of the GPI molecule biosynthesis. (B) In the steady state, PNH erythrocytes suffer from spontaneous (tick-over) complement activation, with consequent intravascular hemolysis through formation of the MAC; exacerbated hemolysis will result from activation of extra complement through the classical pathway. (C) On eculizumab, PNH erythrocytes are protected from hemolysis from the inhibition of C5 cleavage; however, upstream complement activation may lead to C3 opsonization and possible extravascular hemolysis. Here “C3 convertase” represents either the C3bBb complex (alternative pathway) or the C4b2a complex (classical and lectin pathways).
See this image and copyright information in PMC

Comment on

References

    1. Dacie JV. Paroxysmal nocturnal haemoglobinuria. In: Dacie JV, editor. The Haemolytic Anaemias: Drug and Chemical Induced Haemolytic Anaemias, Paroxysmal Nocturnal Haemglobinuria, and Haemolytic Disease of the Newborn. 3rd ed. London: Churchill Livingston; 1999. pp. 139–330.
    1. Luzzatto L, Notaro R. Paroxysmal nocturnal hemoglobinuria. In: Handin RI, Lux SE, Stossel TP, editors. Blood, Principles and Practice of Hematology. 2nd ed. Philadelphia, PA (USA): Lippincot Williams & Wilkins; 2003. pp. 319–34.
    1. Parker C, Omine M, Richards S, Nishimura J, Bessler M, Ware R, et al. Diagnosis and management of paroxysmal nocturnal hemoglobinuria. Blood. 2005;106(12):3699–709. - PMC - PubMed
    1. Marchiafava E. Anemia emolitica cronica con emosiderinuria perpetua. Il Policlinico, Sezione medica. 1928;35:105–17.
    1. Hillmen P, Lewis SM, Bessler M, Luzzatto L, Dacie JV. Natural history of paroxysmal nocturnal hemoglobinuria. N Engl J Med. 1995;333(19):1253–8. - PubMed

MeSH terms