Effect of Eculizumab on Hemolysis and Transfusion Requirements in Patients with Paroxysmal Nocturnal Hemoglobinuria
2004; Massachusetts Medical Society; Volume: 350; Issue: 6 Linguagem: Inglês
10.1056/nejmoa031688
ISSN1533-4406
AutoresPeter Hillmen, Claire Hall, Judith Marsh, Modupe Elebute, Michael P. Bombara, Beth E. Petro, Matthew Cullen, Stephen J. Richards, Scott A. Rollins, Christopher F. Mojcik, Russell P. Rother,
Tópico(s)Blood groups and transfusion
ResumoParoxysmal nocturnal hemoglobinuria (PNH) arises from a somatic mutation of the PIG-A gene in a hematopoietic stem cell and the subsequent production of blood cells with a deficiency of surface proteins that protect the cells against attack by the complement system. We tested the clinical efficacy of eculizumab, a humanized antibody that inhibits the activation of terminal complement components, in patients with PNH.
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