Results 171 to 180 of about 15,382 (226)

Treatment Outcomes of Complement Protein C5 Inhibition in 509 UK Patients with Paroxysmal Nocturnal Hemoglobinuria.

Blood, 2023
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic disorder which occurs on a background of bone marrow failure (BMF). In PNH, chronic intravascular hemolysis causes an increase in morbidity and mortality, mainly due to ...
Richard J. Kelly, Matthew Holt, Jenniner Vidler, L. Arnold, Joanna Large, Briony Forrest, Catherine Barnfield, A. Pike, M. Griffin, T. Munir, P. Muus, S. Nagumantry, Abraham Mullasseril Varghese, John Davies, Roochi Trikha, A. Kulasekararaj, Lindsay D Mitchell, Shreyans Gandhi   +17 more
semanticscholar   +1 more source

Clinical and Molecular Determinants of Clonal Evolution in Aplastic Anemia and Paroxysmal Nocturnal Hemoglobinuria

Journal of Clinical Oncology, 2022
PURPOSE Secondary myeloid neoplasms (sMNs) remain the most serious long-term complications in patients with aplastic anemia (AA) and paroxysmal nocturnal hemoglobinuria (PNH).
C. Gurnari, S. Pagliuca, P. Prata, J. Galimard, L. F. B. Catto, L. Larcher, M. Sébert, V. Allain, B. Patel, Arda Durmaz, A. Pinto, M. Inacio, L. Hernandez, N. Dhédin, S. Caillat-Zucman, E. Clappier, F. Sicre de Fontbrune, M. Voso, V. Visconte, R. Peffault de Latour, J. Soulier, R. Calado, G. Socié, J. Maciejewski   +23 more
semanticscholar   +1 more source

Paroxysmal nocturnal hemoglobinuria

Hématologie, 2013
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disorder of hematopoietic stem cells. PNH is related to a somatic mutation in the phosphatidylinositol glycan class A (PIG-A), X-linked gene, responsible for a deficiency in glycosylphosphatidylinositol-anchored proteins (GPI-AP).
R, Peffault de Latour, Z, Amoura, G, Socié   +2 more
openaire   +2 more sources

Paroxysmal Nocturnal Hemoglobinuria

Journal of Computer Assisted Tomography, 1993
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic disorder, characterized by hemosiderin deposition in the proximal renal tubules.We examined six cases of PNH with MRI including gradient echo imaging.On T2- and T2*-weighted imaging a characteristic hypointense pattern was noted in five of six patients with PNH.
Y O, Tanaka, I, Anno, Y, Itai, T, Abe
openaire   +2 more sources

Paroxysmal nocturnal hemoglobinuria in pregnancy

Acta Obstetricia et Gynecologica Scandinavica, 2003
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia in which a defect of glycophosphatidylinositol (GPI)‐anchored proteins in the cell membrane of bone marrow stem cells leads to increased sensitivity of the red cells to complement, causing intravascular hemolysis and hemoglobinuria.
Line, Bjørge, Peter, Ernst, Kjell O, Haram   +2 more
openaire   +2 more sources

Real‐world experience of pegcetacoplan in paroxysmal nocturnal hemoglobinuria

American journal of hematology/oncology
Pegcetacoplan significantly improves outcomes for patients with paroxysmal nocturnal hemoglobinuria (PNH) experiencing extravascular hemolysis (EVH) on eculizumab, leading to approval in 2021/2022 (USA/Europe).
Morag Griffin, Richard J. Kelly, Isabelle Brindel, Lynda Maafa, Roochi Trikha, P. Muus, T. Munir, A. Varghese, Lindsay D Mitchell, S. Nagumantry, Shreyans Gandhi, A. Pike, A. Kulasekararaj, R. Peffault de Latour   +13 more
semanticscholar   +1 more source

Thrombosis in paroxysmal nocturnal hemoglobinuria

Blood, 2013
Abstract The most frequent and feared complication of paroxysmal nocturnal hemoglobinuria (PNH) is thrombosis. Recent research has demonstrated that the complement and coagulation systems are closely integrated with each influencing the activity of the other to the extent that thrombin itself has recently been shown to activate the ...
Anita, Hill, Richard J, Kelly, Peter, Hillmen   +2 more
openaire   +2 more sources

Thrombosis in Paroxysmal Nocturnal Hemoglobinuria (PNH): From Pathogenesis to Treatment

International Journal of Molecular Sciences
Paroxysmal Nocturnal Hemoglobinuria (PNH) constitutes a rare bone marrow failure syndrome characterized by hemolytic anemia, thrombotic events (TEs), and bone marrow aplasia of variable degrees.
Styliani Kokoris, Antri Polyviou, P. Evangelidis, E. Grouzi, S. Valsami, Konstantinos Tragiannidis, A. Gialeraki, Dimitrios A. Tsakiris, E. Gavriilaki   +8 more
semanticscholar   +1 more source

The syndrome of paroxysmal nocturnal hemoglobinuria

The American Journal of Medicine, 1964
Abstract The case histories and investigations are presented of four patients with paroxysmal nocturnal hemoglobinuria who satisfy the diagnostic criteria of abnormal erythrocyte acid hemolysis, positive intravascular hemolysis and low erythrocyte acetylcholinesterase. The clinical presentations of these patients ranged from a young man with a classic
R W, BEAL, H, KRONENBERG, B G, FIRKIN
openaire   +2 more sources