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Titolo:
Detection of GPI-anchored protein-deficient cells in patients with aplastic anaemia and evidence for clonal expansion during the clinical course
Autore:
Kawaguchi, K; Wada, H; Mori, A; Takemoto, Y; Kakishita, E; Kanamaru, A;
Indirizzi:
Hyogo Coll Med, Dept Internal Med 2, Nishinomiya, Hyogo 6638131, Japan Hyogo Coll Med Nishinomiya Hyogo Japan 6638131 miya, Hyogo 6638131, Japan Kinki Univ, Sch Med, Dept Internal Med 3, Osaka 589, Japan Kinki Univ Osaka Japan 589 ch Med, Dept Internal Med 3, Osaka 589, Japan
Titolo Testata:
BRITISH JOURNAL OF HAEMATOLOGY
fascicolo: 1, volume: 105, anno: 1999,
pagine: 80 - 84
SICI:
0007-1048(199904)105:1<80:DOGPCI>2.0.ZU;2-O
Fonte:
ISI
Lingua:
ENG
Soggetto:
PAROXYSMAL-NOCTURNAL HEMOGLOBINURIA; DECAY-ACCELERATING FACTOR; FLOW CYTOMETRIC ANALYSIS; PIG-A GENE; HEMATOPOIETIC PROGENITORS; NORMAL INDIVIDUALS; SOMATIC MUTATIONS; PATHOGENETIC LINK; ANEMIA PATIENTS; BONE-MARROW;
Keywords:
aplastic anaemia; GPI-anchored protein; paroxysmal nocturnal haemoglobinuria; PIG-A; clonal expansion;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
17
Recensione:
Indirizzi per estratti:
Indirizzo: Wada, H Hyogo638131,ed, Dept Internal Med 2, 1-1 Mukogawa Cho, Nishinomiya, Hyogo 6 Hyogo Coll Med 1-1 Mukogawa Cho Nishinomiya Hyogo Japan 6638131 6
Citazione:
K. Kawaguchi et al., "Detection of GPI-anchored protein-deficient cells in patients with aplastic anaemia and evidence for clonal expansion during the clinical course", BR J HAEM, 105(1), 1999, pp. 80-84

Abstract

The incidence of patients with aplastic anaemia (AA) who show a deficiencyof glycosylphosphatidylinositol (GPI)-anchored proteins on their peripheral blood (PB) or bone marrow (BM) cells is higher than that previously reported. We analysed the expression of CD55 or CD59 on PB and BM cells and those of colonies and bursts formed in cultures with marrow cells from AA patients. 4/21 (19%) AA cases later developed paroxysmal nocturnal haemoglobinuria (PNH), 7/17 (41.2%) AA cases showed a subpopulation without GPI-anchoredproteins. The defect characteristic for PNH was observed only on the colonies/bursts but not on the PB or BM cells in three cases. We found the affected colonies/bursts in cultures using thawed marrow cells which had been cryopreserved at the diagnosis of aplasia in one patient who developed PNH 3 years later. Two different mutations of the PIG-A gene were found in the colonies/bursts at the time of AA. The nucleotide sequences were identical between the colonies/bursts at the time of AA and those after the transition to PNH. However, one of the mutations was detected only at the haemopoieticprogenitor level but not in PB granulocytes. There may be latent subcloneswith PNH abnormalities which could expand to the level of clinical detection, or which do not progress and remain indolent for a relatively long time, implying the different extents of clonal expansion among the affected progenitors.

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Documento generato il 27/09/20 alle ore 00:21:39