Clinical and biologic diversity of leukemias occurring in patients with mastocytosis

Citation
Wr. Sperr et al., Clinical and biologic diversity of leukemias occurring in patients with mastocytosis, LEUK LYMPH, 37(5-6), 2000, pp. 473-486
Citations number
106
Language
INGLESE
art.tipo
Review
Categorie Soggetti
Hematology,"Onconogenesis & Cancer Research
Journal title
LEUKEMIA & LYMPHOMA
ISSN journal
1042-8194 → ACNP
Volume
37
Issue
5-6
Year of publication
2000
Pages
473 - 486
Database
ISI
SICI code
1042-8194(200005)37:5-6<473:CABDOL>2.0.ZU;2-9
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 myel ogenous malignancies. In a significant proportion of cases. myeloid leukemi as occur. Using conventional criteria, such leukemias resemble acute mlyelo id leukemia (AML), chronic myeloid leukemia (CML), or myelomonocytic leukem ia (CMML). Mast cell leukemia (MCL) may also occur. Myeloid leukemias (AML, CML, CMML) can develop in indolent or aggressive mastocytosis (skin lesion s present or absent) with a variable prephase of MG disease. By contrast, M CL (typically without skin lesions) often develops on a "de novo" basis, an d, if at all recognized, a prephase resembling (malignant) mastocytosis, is short. MCL differs from myeloid leukemias (AML, CML, CMML) by morphologic and phenotypic cellular characteristics. In fact. MCL are strongly tryptase -positive, c-kit-positive, myeloperoxidase (MPO) -negative neoplasms with v ariable metachromasia and chloroacetate esterase expression, whereas an MPO -positive, tryptase-negative phenotype supports the diagnosis of a myeloid non-MG lineage disease. Thus, MCL, but also myeloid non-MC lineage leukemia s can develop in patients with (systemic) mastocytosis. Little is known, ho wever, about the pathophysiologic basis of co-evolution. In the present art icle, the concomitant occurrence of mastocytosis and leukemia is discussed in the light of the literature and of concepts proposed to explain the biol ogic basis of this phenomenon.