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Titolo:
Clinical and biologic diversity of leukemias occurring in patients with mastocytosis
Autore:
Sperr, WR; Horny, HP; Lechner, K; Valent, P;
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
Titolo Testata:
LEUKEMIA & LYMPHOMA
fascicolo: 5-6, volume: 37, anno: 2000,
pagine: 473 - 486
SICI:
1042-8194(200005)37:5-6<473:CABDOL>2.0.ZU;2-9
Fonte:
ISI
Lingua:
ENG
Soggetto:
MAST-CELL DISEASE; BLOOD MONONUCLEAR-CELLS; ACUTE MYELOID-LEUKEMIA; C-KIT MUTATION; CHRONIC MYELOGENOUS LEUKEMIA; SYSTEMIC MASTOCYTOSIS; BONE-MARROW; PERIPHERAL-BLOOD; MYELOPROLIFERATIVE DISORDERS; URTICARIA PIGMENTOSA;
Keywords:
mast cells; AMI; CML; CMML; c-kit; tryptase;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
106
Recensione:
Indirizzi per estratti:
Indirizzo: Sperr, WR Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Wahringer Gurtel 18-20, A-1090 Vienna, Austria Univ Vienna Wahringer Gurtel 18-20 Vienna Austria A-1090 ustria
Citazione:
W.R. Sperr et al., "Clinical and biologic diversity of leukemias occurring in patients with mastocytosis", LEUK LYMPH, 37(5-6), 2000, pp. 473-486

Abstract

Patients with systemic mast cell (MC) disease, but not those with cutaneous mastocytosis, are at a high risk (10-30%) to develop life-threatening myelogenous malignancies. In a significant proportion of cases. myeloid leukemias occur. Using conventional criteria, such leukemias resemble acute mlyeloid leukemia (AML), chronic myeloid leukemia (CML), or myelomonocytic leukemia (CMML). Mast cell leukemia (MCL) may also occur. Myeloid leukemias (AML, CML, CMML) can develop in indolent or aggressive mastocytosis (skin lesions present or absent) with a variable prephase of MG disease. By contrast, MCL (typically without skin lesions) often develops on a "de novo" basis, and, if at all recognized, a prephase resembling (malignant) mastocytosis, is short. MCL differs from myeloid leukemias (AML, CML, CMML) by morphologicand phenotypic cellular characteristics. In fact. MCL are strongly tryptase-positive, c-kit-positive, myeloperoxidase (MPO) -negative neoplasms with variable metachromasia and chloroacetate esterase expression, whereas an MPO-positive, tryptase-negative phenotype supports the diagnosis of a myeloidnon-MG lineage disease. Thus, MCL, but also myeloid non-MC lineage leukemias can develop in patients with (systemic) mastocytosis. Little is known, however, about the pathophysiologic basis of co-evolution. In the present article, the concomitant occurrence of mastocytosis and leukemia is discussedin the light of the literature and of concepts proposed to explain the biologic basis of this phenomenon.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 30/09/20 alle ore 00:52:25