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

Animesh D Pardanani, Ken Hong Lim, Terra L. Lasho, Christy Finke, Rebecca F. McClure, Chin Yang Li, Ayalew Tefferi

Research output: Contribution to journalArticle

111 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)3769-3772
Number of pages4
JournalBlood
Volume114
Issue number18
DOIs
StatePublished - Oct 29 2009

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Systemic Mastocytosis
Health
Leukemia, Myelomonocytic, Chronic
Neoplasms
Leukemia
Myelodysplastic Syndromes
Eosinophilia
Cell Lineage
Life Expectancy

ASJC Scopus subject areas

  • Hematology
  • Biochemistry
  • Cell Biology
  • Immunology

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Prognostically relevant breakdown of 123 patients with systemic mastocytosis associated with other myeloid malignancies. / Pardanani, Animesh D; Lim, Ken Hong; Lasho, Terra L.; Finke, Christy; McClure, Rebecca F.; Li, Chin Yang; Tefferi, Ayalew.

In: Blood, Vol. 114, No. 18, 29.10.2009, p. 3769-3772.

Research output: Contribution to journalArticle

Pardanani, Animesh D ; Lim, Ken Hong ; Lasho, Terra L. ; Finke, Christy ; McClure, Rebecca F. ; Li, Chin Yang ; Tefferi, Ayalew. / Prognostically relevant breakdown of 123 patients with systemic mastocytosis associated with other myeloid malignancies. In: Blood. 2009 ; Vol. 114, No. 18. pp. 3769-3772.
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