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Titolo:
Cytogenetic and morphological abnormalities in paroxysmal nocturnal haemoglobinuria
Autore:
Araten, DJ; Swirsky, D; Karadimitris, A; Notaro, R; Nafa, K; Bessler, M; Thaler, HT; Castro-Malaspina, H; Childs, BH; Boulad, F; Weiss, M; Anagnostopoulos, N; Kutlar, A; Savage, DG; Maziarz, RT; Jhanwar, S; Luzzatto, L;
Indirizzi:
Ist Nazl Ric Canc, IST, Genoa, Italy Ist Nazl Ric Canc Genoa ItalyIst Nazl Ric Canc, IST, Genoa, Italy Mem Sloan Kettering Canc Ctr, Dept Human Genet, New York, NY 10021 USA MemSloan Kettering Canc Ctr New York NY USA 10021 New York, NY 10021 USA Mem Sloan Kettering Canc Ctr, Dept Hematol, New York, NY 10021 USA Mem Sloan Kettering Canc Ctr New York NY USA 10021 New York, NY 10021 USA Leeds Gen Infirm, Dept Haematol, Haematol Malignancy Diag Serv, Leeds, W Yorkshire, England Leeds Gen Infirm Leeds W Yorkshire England , Leeds, W Yorkshire, England Mem Sloan Kettering Canc Ctr, Dept Biostat, New York, NY 10021 USA Mem Sloan Kettering Canc Ctr New York NY USA 10021 New York, NY 10021 USA Mem Sloan Kettering Canc Ctr, Dept Pediat, New York, NY 10021 USA Mem Sloan Kettering Canc Ctr New York NY USA 10021 New York, NY 10021 USA G Genimatas Gen Hosp, Dept Haematol, Athens, Greece G Genimatas Gen Hosp Athens Greece Hosp, Dept Haematol, Athens, Greece Med Coll Georgia, Dept Med, Augusta, GA 30912 USA Med Coll Georgia Augusta GA USA 30912 ia, Dept Med, Augusta, GA 30912 USA Columbia Univ, Div Hematol Oncol, New York, NY USA Columbia Univ New YorkNY USA Univ, Div Hematol Oncol, New York, NY USA Oregon Hlth Sci Univ, Portland, OR 97201 USA Oregon Hlth Sci Univ Portland OR USA 97201 i Univ, Portland, OR 97201 USA
Titolo Testata:
BRITISH JOURNAL OF HAEMATOLOGY
fascicolo: 2, volume: 115, anno: 2001,
pagine: 360 - 368
SICI:
0007-1048(200111)115:2<360:CAMAIP>2.0.ZU;2-N
Fonte:
ISI
Lingua:
ENG
Soggetto:
BONE-MARROW TRANSPLANTATION; PIG-A GENE; APLASTIC-ANEMIA; IMMUNOSUPPRESSIVE THERAPY; MYELODYSPLASTIC SYNDROMES; REFRACTORY-ANEMIA; SOMATIC MUTATIONS; SEPARATE CLONE; HEMOGLOBINURIA; EVOLUTION;
Keywords:
paroxysmal nocturnal haemoglobinuria; chromosomal abnormalities; myelodysplastic syndrome; aplastic anaemia; bone marrow morphology;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
54
Recensione:
Indirizzi per estratti:
Indirizzo: Luzzatto, L Ist Nazl Ric Canc, IST, Largo Rosanna Benzi 10, Genoa, Italy Ist Nazl Ric Canc Largo Rosanna Benzi 10 Genoa Italy , Italy
Citazione:
D.J. Araten et al., "Cytogenetic and morphological abnormalities in paroxysmal nocturnal haemoglobinuria", BR J HAEM, 115(2), 2001, pp. 360-368

Abstract

Paroxysmal nocturnal haemoglobinuria (PNH) is characterized by the expansion of a haematopoietic stem cell clone with a PIG-A mutation (the PNH clone) in an environment in which normal stem cells are lost or failing: it has been hypothesized that this abnormal marrow environment provides a relativeadvantage to the PNH clone. In patients with PNH, generally, the karyotypeof bone marrow cells has been reported to be normal, unlike in myelodysplastic syndrome (MDS), another clonal condition in which cytogenetic abnormalities are regarded as diagnostic. In a retrospective review of 46 patients with a PNH clone. we found a karyotypic abnormality in 11 (24%). Upon follow-up, the proportion of cells with abnormal karyotype decreased significantly in seven of these 11 patients. Abnormal morphological bone marrow features reminiscent of MDS were common in PNH, regardless of the karyotype. However, none of our patients developed excess blasts or leukaemia. We concludethat in patients with PNH cytogenetically abnormal clones are not necessarily malignant and may not be predictive of evolution to leukaemia.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 29/09/20 alle ore 23:58:27