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Ahemolytic PNH (white cell PNH): Clinical features and implications of a distinct phenotype of paroxysmal nocturnal haemoglobinuria.

Authors :
Tombul, Zehra
Bahaj, Waled
Ozturk, Merve
Patel, Bhavisha
Toprak, Ahmet
Ibrahim, Ibrahim
Chen, Weina
Fuda, Franklin
Ogbue, Olisaemeka D.
Gurnari, Carmelo
Parker, Charles
Young, Neal S.
Maciejewski, Jaroslaw P.
Duran, Munevver
Bat, Taha
Source :
British Journal of Haematology. May2024, Vol. 204 Issue 5, p2121-2124. 4p.
Publication Year :
2024

Abstract

The article explores a specific type of paroxysmal nocturnal haemoglobinuria (PNH) known as ahemolytic PNH or white cell PNH. Unlike typical PNH, this subtype does not exhibit signs of hemolysis but is characterized by bone marrow failure and thrombophilia. The document suggests that this subtype may be caused by a PIGA mutation in a committed progenitor cell rather than a stem cell. The authors recommend considering screening for ahemolytic PNH in cases of thromboembolic events and bone marrow failure without hemolysis. [Extracted from the article]

Details

Language :
English
ISSN :
00071048
Volume :
204
Issue :
5
Database :
Academic Search Index
Journal :
British Journal of Haematology
Publication Type :
Academic Journal
Accession number :
177190955
Full Text :
https://doi.org/10.1111/bjh.19395