Results 71 to 80 of about 7,137 (182)
British Journal of Haematology, Volume 209, Issue 1, Page 324-328, July 2026.
K. Tozatto‐Maio +16 more
wiley +1 more source
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic stem cell disease caused by abnormal expression of glycosylphosphatidylinositol (GPI) on the cell membrane due to mutations in the phosphatidylinositol glycan class A(PIGA ...
CHEN Miao +26 more
doaj +1 more source
Pathogenesis of paroxysmal nocturnal hemoglobinuria
Abstract Paroxysmal nocturnal hemoglobinuria (PNH) is a nonmalignant clonal hematopoietic disorder. There are 2 components to the pathogenesis of PNH: (1) a mutant stem cell and (2) expansion of the mutant clone. Component 1 is straightforward: there is almost always an inactivating somatic mutation of the X-linked ...
Lucio Luzzatto, Shinji Nakao
openaire +2 more sources
Evolutionary dynamics of paroxysmal nocturnal hemoglobinuria
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal blood disorder characterized by hemolysis and a high risk of thrombosis, that is due to a deficiency in several cell surface proteins that prevent complement activation. Its origin has been traced to a somatic mutation in the PIG-A gene within hematopoietic stem cells (HSC).
Nathaniel Mon Père +3 more
openaire +6 more sources
Key Clinical message Eculizumab in pregnancy has been reported to be effective in improving outcomes in patients with paroxysmal nocturnal hemoglobinuria. However, a cesarean section may result in surgery‐triggered hemolysis.
Dongping Li +8 more
doaj +1 more source
Key Clinical Message Haploidentical hematopoietic stem cell transplantation (HSCT) with posttransplant cyclophosphamide is an alternative treatment for aplastic anemia–paroxysmal nocturnal hemoglobinuria (PNH) syndrome with poor prognostic factors ...
Kazuki Sakurai +6 more
doaj +1 more source
Necrotizing Fasciitis in Paroxysmal Nocturnal Hemoglobinuria
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, progressive, and life-threatening hematopoietic stem cell disorder characterized by complement-mediated intravascular hemolysis and a prothrombotic state.
Pusem Patir +8 more
doaj +1 more source
Paroxysmal nocturnal hemoglobinuria and thrombosis
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired clonal hematopoiesis disorder caused by a somatic mutation in the PIGA (phosphatidylinositol glycan, class A) gene, resulting in deficiency of glycosylphosphatidylinositol (GPI)anchored ...
A. R. Khisamieva +12 more
doaj +1 more source
A Case Presenting with Acute Kidney Injury and Paroxysmal Nocturnal Hemoglobinuria
There may be different clinical presentations of kidney involvement in the course of paroxysmal nocturnal hemoglobinuria, within a spectrum of acute kidney injury, generally due to hemolysis, to chronic kidney disease.
Ezgi ÇOŞKUN YENİGÜN +6 more
doaj
Prathit A Kulkarni1, Vahid Afshar-Kharghan21Baylor College of Medicine, Houston, Texas, USA; 2The University of Texas, M.D. Anderson Cancer Center, Houston, Texas, USAAbstract: The complement system is an important part of innate immunity; however, as ...
Prathit A Kulkarni +1 more
doaj

