paroxysmal nocturnal hemoglobinuria (PNH) | Page 21 | Aplastic Anemia and MDS International Foundation

paroxysmal nocturnal hemoglobinuria (PNH)

Guidelines for the diagnosis and monitoring of paroxysmal nocturnal hemoglobinuria and related disorders by flow cytometry.

Author(s): 
Borowitz MJ, Craig FE, Digiuseppe JA, Illingworth AJ, Rosse W, Sutherland DR, Wittwer CT, Richards SJ; Clinical Cytometry Society
Primary Author: 
Borowitz MJ
Journal Title: 
Cytometry B Clin Cytom
Original Publication Date: 
Jul 2010

BACKGROUND:

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematopoietic stem cell disorder

Long-term effect of the complement inhibitor eculizumab on kidney function in patients with paroxysmal nocturnal hemoglobinuria.

Author(s): 
Hillmen P, Elebute M, Kelly R, Urbano-Ispizua A, Hill A, Rother RP, Khursigara G, Fu CL, Omine M, Browne P, Rosse W.
Primary Author: 
Hillmen P
Journal Title: 
Am J Hematol
Original Publication Date: 
Aug 2010

Long term treatment with eculizumab in paroxysmal nocturnal hemoglobinuria: sustained efficacy and improved survival.

Author(s): 
Kelly RJ, Hill A, Arnold LM, Brooksbank GL, Richards SJ, Cullen M, Mitchell LD, Cohen DR, Gregory WM, Hillmen P.
Primary Author: 
Kelly RJ
Journal Title: 
Blood
Original Publication Date: 
Apr 2011

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic disorder with increased mortality and morbidity due to intense intravascular

Loss of expression of neutrophil proteinase-3: a contributory factor in thrombotic risk in paroxysmal nocturnal hemoglobinuria.

Author(s): 
Jankowska AM, Szpurka H, Calabro M, Mohan S, Schade AE, Clemente M, Silverstein RL, Maciejewski JP
Primary Author: 
Jankowska AM
Journal Title: 
Haematologica
Original Publication Date: 
May 2011

Background: A deficiency of specific glycosylphosphatidyl inositol-anchored proteins in paroxysmal nocturnal hemoglobinuria may be responsible for most of the clinical features of this disease, but some functional consequences may be indirect. For example, the absence of certain glycosylphosphatidyl