Prognostically relevant breakdown of 123 patients with systemic mastocytosis associated with other myeloid malignancies.

The prognostic heterogeneity of the World Health Organization category of "systemic mastocytosis with associated clonal hematologic nonmast cell lineage disease" (SM-AHNMD) has not been systematically validated by primary data. Among 138 consecutive cases with SM-AHNMD, 123 (89%) had associated myeloid neoplasm: 55 (45%) myeloproliferative neoplasm (SM-MPN), 36 (29%) chronic myelomonocytic leukemia, 28 (23%) myelodysplastic syndrome (SM-MDS), and 4 (3%) acute leukemia. Of the myeloid subgroups, SM-MPN displayed a 2- to 3-fold better life expectancy (P = .003), whereas leukemic transformation was more frequent in SM-MDS (29%; P = .02). The presence of eosinophilia, although prevalent (34%), was prognostically neutral, and the overall results were not affected by exclusion of FIP1L1-PDGFRA-positive cases. We conclude that it is clinically more useful to consider specific entities, such as SM-MPN, systemic mastocytosis with chronic myelomonocytic leukemia, SM-MDS, and systemic mastocytosis with-acute leukemia, rather than their broad reference as SM-AHNMD.

[1]  A. Tefferi,et al.  Systemic mastocytosis in 342 consecutive adults: survival studies and prognostic factors. , 2009, Blood.

[2]  N. Shinton.  WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues , 2007 .

[3]  L. Weiss,et al.  Distinct clonal origins of systemic mastocytosis and associated B-cell lymphoma. , 2007, Leukemia research.

[4]  M. Triggiani,et al.  Standards and standardization in mastocytosis: Consensus Statements on Diagnostics, Treatment Recommendations and Response Criteria , 2007, European journal of clinical investigation.

[5]  A. Tefferi,et al.  Clinical correlates of JAK2V617F allele burden in essential thrombocythemia , 2007, Cancer.

[6]  A. Órfão,et al.  KIT mutation in mast cells and other bone marrow hematopoietic cell lineages in systemic mast cell disorders: a prospective study of the Spanish Network on Mastocytosis (REMA) in a series of 113 patients. , 2006, Blood.

[7]  K. Sotlar,et al.  Systemic mastocytosis with associated clonal haematological non-mast cell lineage diseases: a histopathological challenge , 2004, Journal of Clinical Pathology.

[8]  P. Lipsky,et al.  Evidence for the involvement of a hematopoietic progenitor cell in systemic mastocytosis from single-cell analysis of mutations in the c-kit gene. , 2002, Blood.

[9]  L. Scott,et al.  Analysis of the surface expression of c-kit and occurrence of the c-kit Asp816Val activating mutation in T cells, B cells, and myelomonocytic cells in patients with mastocytosis. , 2000, Experimental Hematology.

[10]  J. Bernhard,et al.  Mastocytosis , 1997, The Lancet.

[11]  E. Bergstralh,et al.  Significance of systemic mast cell disease with associated hematologic disorders , 1988, Cancer.