The Role of Hemolysis
PNH is an acquired genetic deficiency characterized clinically by chronic hemolysis.1-2
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- Normal red blood cells (RBCs) express a protein that inhibits terminal complement (CD59)1,3
- The absence of this protein leads to chronic complement-mediated lysis of RBCs1,3,4
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References:
1. Rosse WF, Hillmen P, Schrieber AD. Immune-mediated hemolytic anemia. Hematology. (Am Soc Hematol Educ Program) January 2004:48-62.
2. Hillmen P, Lewis SM, Bessler M, Luzzatto L, Dacie JV. Natural history of paroxysmal nocturnal hemoglobinuria. N Engl J Med. 1995;333:1253-1258.
3. Johnson RJ, Hillmen P. Paroxysmal nocturnal hemoglobinuria: nature's gene therapy? J Clin Pathol Mol Pathol. 2992;55:145-152.
4. Luzzatto L, Gianfaldoni G. Recent advances in biological and clinical aspects of paroxysmal nocturnal hemoglobinuria. Int J Hematol. 2006;84:104-112.
5. Parker C, Omine M, Richards S, et al. Diagnosis and management of paroxysmal nocturnal hemoglobinuria. Blood. 2005;106:3699-3709.
6. Brodsky RA. Paroxysmal nocturnal hemoglobinuria. In: Hoffman R, Benz EJ, Shattil SJ, et al. eds. Hematology. 4th ed. Philadelphia, PA: Elsevier Churchill Livingstone. 2005:419-427.
7. Rother RP, Bell L, Hillmen P, Gladwin MT. The clinical sequelae of intravascular hemolysis and extracellular plasma hemoglobin. JAMA. 2005;293:1653-1662