A cohort study of the nature of paroxysmal nocturnal hemoglobinuria clones and PIG-A mutations in patients with aplastic anemia

European Journal of Haematology
Wanchai WanachiwanawinTaroh Kinoshita

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is characterized by the clonal expansion of blood cells, which are deficient in glycosylphosphatidylinositol anchored proteins (GPI-APs). As PNH frequently occurs during the clinical course of acquired aplastic anemia (AA), it is likely that a process inducing bone marrow failure in AA is responsible for the selection of GPI-AP deficient blood cells or PNH clone. To explore the nature and mutation of a PNH clone in AA. We performed regular repeated flow cytometric analyses of CD59 expression on peripheral blood cells from a cohort of 32 patients with AA. Mutation of phosphatidylinositol glycan class A (PIG-A) was also studied. Fifty-one episodes of occurrences of CD59 negative granulocytes out of a total cohort 167 flow cytometric analyses (31%) were observed in 22 patients (69%). CD59 negative erythrocytes were less apparent than the granulocytes. Repeated occurrences of PNH clones were observed in 16 patients. Most of the emerging PNH clones were transient in nature. They were more frequently detected during episodes of lower white blood cell and platelet counts. Persistence and expansion of the GPI-AP deficient blood cell populations to the level of clinical PNH were seen in only fou...Continue Reading

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Citations

Jul 15, 2015·American Journal of Blood Research·Fahri SahinGuray Saydam
Nov 5, 2016·Indian Journal of Hematology & Blood Transfusion : an Official Journal of Indian Society of Hematology and Blood Transfusion·Amrallah A MohammedAyman A Rasmy
Jul 20, 2012·Pediatric Blood & Cancer·Kathryn S SuttonPeter Kurre
Aug 24, 2010·Histopathology·Roos J Leguit, Jan G van den Tweel
Nov 15, 2013·Cytometry. Part B, Clinical Cytometry·Azra RazaAndrea Illingworth
Nov 6, 2013·International Journal of Laboratory Hematology·K CanalejoM Aixalá
Aug 7, 2007·International Journal of Hematology·Tatsuya Kawaguchi, Hideki Nakakuma
Jun 19, 2018·PLoS Computational Biology·Nathaniel Mon PèreDavid Dingli
Oct 28, 2017·Indian Journal of Hematology & Blood Transfusion : an Official Journal of Indian Society of Hematology and Blood Transfusion·Prabhu ManivannanHara Prasad Pati

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