[Novel therapeutics for paroxysmal nocturnal hemoglobinuria].

Glycosylphosphatidylinositols Hematopoietic Stem Cells Hemoglobinuria, Paroxysmal / drug therapy Hemolysis Humans Mannosyltransferases Quality of Life
Paroxysmal nocturnal hemoglobinuria Proximal complement Recycling antibody Terminal complement

Journal

[Rinsho ketsueki] The Japanese journal of clinical hematology
ISSN: 0485-1439
Titre abrégé: Rinsho Ketsueki
Pays: Japan
ID NLM: 2984782R

Informations de publication

Date de publication:
2021
Historique:
entrez: 10 6 2021
pubmed: 11 6 2021
medline: 12 6 2021
Statut: ppublish

Résumé

Following the reports of paroxysmal nocturnal hemoglobinuria (PNH) due to PIGT and PIGB gene mutations, the definition of PNH was changed to a hematopoietic stem cell disease with complement-mediated intravascular hemolysis as a result of clonal expansion of hematopoietic stem cells with mutations in genes involved in glycosylphosphatidylinositol (GPI) anchor synthesis, including PIGA. Treatment with eculizumab (Soliris

Identifiants

DOI: 10.11406/rinketsu.62.463 PMID: 34108330
pubmed: 34108330
doi: 10.11406/rinketsu.62.463
doi:

Substances chimiques

Glycosylphosphatidylinositols 0
Mannosyltransferases EC 2.4.1.-
PIGB protein, human EC 2.4.1.-

Types de publication

Journal Article

Langues

jpn

Sous-ensembles de citation

IM

Pagination

463-471

Auteurs

Jun-Ichi Nishimura (JI)

Department of Hematology and Oncology, Osaka University Graduate School of Medicine.

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Classifications MeSH

questionsmedicales.fr › Lipides › Lipides membranaires › Phospholipides › Glycérophosphate › Acides phosphatidiques › Glycérophospholipides › Phosphatidyl inositols › Glycosylphosphatidylinositols › [Novel therapeutics for paroxysmal nocturnal...
questionsmedicales.fr › Systèmes sanguin et immunitaire › Système hématopoïétique › Cellules de la moelle osseuse › Cellules souches hématopoïétiques › [Novel therapeutics for paroxysmal nocturnal...
questionsmedicales.fr › Hémopathies et maladies lymphatiques › Hémopathies › Maladies de la moelle osseuse › Syndromes myélodysplasiques › Hémoglobinurie paroxystique › [Novel therapeutics for paroxysmal nocturnal...
questionsmedicales.fr › Phénomènes du système immunitaire › Réaction antigène-anticorps › Hémolyse › [Novel therapeutics for paroxysmal nocturnal...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › [Novel therapeutics for paroxysmal nocturnal...
questionsmedicales.fr › Enzymes et coenzymes › Enzymes › Transferases › Glycosyltransferase › Hexosyltransferases › Mannosyltransferases › [Novel therapeutics for paroxysmal nocturnal...
questionsmedicales.fr › Environnement et santé publique › Santé publique › Mesures épidémiologiques › Démographie › État de santé › Qualité de vie › [Novel therapeutics for paroxysmal nocturnal...