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Titolo:
Myelomastocytic overlap syndromes: biology, criteria, and relationship to mastocytosis
Autore:
Valent, P; Sperr, WR; Samorapoompichit, P; Geissler, K; Lechner, K; Horny, HP; Bennett, JM;
Indirizzi:
Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, A-1090 Vienna, Austria Univ Vienna Vienna Austria A-1090 Internal Med 1, A-1090 Vienna, Austria Univ Vienna, Dept Histol & Embryol, A-1010 Vienna, Austria Univ Vienna Vienna Austria A-1010 stol & Embryol, A-1010 Vienna, Austria Med Univ Lubeck, Inst Pathol, D-23538 Lubeck, Germany Med Univ Lubeck Lubeck Germany D-23538 t Pathol, D-23538 Lubeck, Germany Univ Rochester, Med Oncol Unit, Rochester, NY 14627 USA Univ Rochester Rochester NY USA 14627 Oncol Unit, Rochester, NY 14627 USA
Titolo Testata:
LEUKEMIA RESEARCH
fascicolo: 7, volume: 25, anno: 2001,
pagine: 595 - 602
SICI:
0145-2126(200107)25:7<595:MOSBCA>2.0.ZU;2-5
Fonte:
ISI
Lingua:
ENG
Soggetto:
ACUTE MYELOID-LEUKEMIA; STEM-CELL FACTOR; BLOOD MONONUCLEAR-CELLS; MARROW MAST-CELLS; C-KIT; BONE-MARROW; MYELODYSPLASTIC SYNDROMES; SYSTEMIC MASTOCYTOSIS; ACTIVATING MUTATION; CATALYTIC DOMAIN;
Keywords:
myelodysplasia; overlap syndromes; mastocytosis; mast cells; c-kit; tryptase;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
45
Recensione:
Indirizzi per estratti:
Indirizzo: Valent, P Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Waehringer Guertel 18-20, A-1090 Vienna, Austria Univ Vienna Waehringer Guertel 18-20 Vienna Austria A-1090 tria
Citazione:
P. Valent et al., "Myelomastocytic overlap syndromes: biology, criteria, and relationship to mastocytosis", LEUK RES, 25(7), 2001, pp. 595-602

Abstract

Although mast cells (MC) appear to be myeloid cells, MC lineage involvement in myelogenous malignancies has been described only rarely. Based on clonal evolution, biology of afflicted cells, and disease criteria, three majorgroups of patients have been recognized: The first meets criteria for bothdiagnoses 'systemic mastocytosis' and 'associated hematologic clonal non-mast cell lineage disease (AHNMD)'. In such patients, myeloproliferative (MPS) or myelodysplastic syndromes (MDS), or acute myeloid leukemia (AML) is diagnosed apart from mastocytosis. In a second group of patients, large numbers of very immature MC-lineage cells (metachromatically granulated blast-like cells) are detectable, but the criteria to diagnose mastocytosis are not met. These patients have advanced myeloid neoplasms (MDS or MPS with blast cell increase, or AML) and variably suffer from mediator-related symptoms(flush, GI-tract ulcer, diarrhoea, coagulopathy). In some cases, the disease mimics mast cell or basophilic leukemia. In contrast to basophilic leukemia, however, the metachromatic cells are strongly KIT + and tryptase +. Incontrast to true mast cell leukemia (MCL), MC do not form multifocal denseinfiltrates in the bone marrow. Also, MC lack CD2 and CD25, and the C-KIT mutation Asp-816-Val. We propose the term 'myelomastocytic leukemia' or 'myelodysplastic mast cell syndrome' for these cases. In a third group of patients, myeloid neoplasms (MDS, MPS, AML) show constitutive expression of MC-associated antigens (tryptase, histamine) or mastocytosis-related gene defects (mutated C-KIT) without significant increase in metachromatic cells or criteria of mastocytosis. Whether these neoplasms display aberrant gene expression (or gene defects) or represent 'pre-pre-mast cell leukemias', remains unknown. (C) 2001 Elsevier Science Ltd. All rights reserved.

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Documento generato il 19/09/20 alle ore 17:34:20