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

Blood. 2009 Oct 29;114(18):3769-72. doi: 10.1182/blood-2009-05-220145. Epub 2009 Aug 27.

Abstract

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.

Publication types

  • Clinical Trial

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Eosinophilia / complications
  • Eosinophilia / diagnosis
  • Eosinophilia / epidemiology
  • Eosinophilia / genetics
  • Eosinophilia / metabolism
  • Female
  • Hematologic Neoplasms / complications
  • Hematologic Neoplasms / diagnosis
  • Hematologic Neoplasms / epidemiology*
  • Hematologic Neoplasms / genetics
  • Hematologic Neoplasms / metabolism
  • Humans
  • Male
  • Mastocytosis, Systemic / complications
  • Mastocytosis, Systemic / diagnosis
  • Mastocytosis, Systemic / epidemiology*
  • Mastocytosis, Systemic / genetics
  • Mastocytosis, Systemic / metabolism
  • Middle Aged
  • Neoplasms, Second Primary / diagnosis
  • Neoplasms, Second Primary / epidemiology*
  • Neoplasms, Second Primary / genetics
  • Neoplasms, Second Primary / metabolism
  • Oncogene Proteins, Fusion / genetics
  • Oncogene Proteins, Fusion / metabolism
  • Prognosis
  • Receptor, Platelet-Derived Growth Factor alpha / genetics
  • Receptor, Platelet-Derived Growth Factor alpha / metabolism
  • mRNA Cleavage and Polyadenylation Factors / genetics
  • mRNA Cleavage and Polyadenylation Factors / metabolism

Substances

  • Oncogene Proteins, Fusion
  • mRNA Cleavage and Polyadenylation Factors
  • FIP1L1-PDGFRA fusion protein, human
  • Receptor, Platelet-Derived Growth Factor alpha