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 Blood, 29 October 2009, Vol. 114, No. 18, pp. 3769-3772.
Prepublished online as a Blood First Edition Paper on August 27, 2009; DOI 10.1182/blood-2009-05-220145.

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CLINICAL TRIALS AND OBSERVATIONS

Brief report

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

Animesh Pardanani1, Ken-Hong Lim1,2, Terra L. Lasho1, Christy Finke1, Rebecca F. McClure3, Chin-Yang Li3, and Ayalew Tefferi1

1 Division of Hematology, Mayo Clinic, Rochester, MN; 2 Division of Hematology-Oncology, Mackay Memorial Hospital, and Mackay Medicine, Nursing and Management College, Taipei, Taiwan, Republic of China; and 3 Division of Hematopathology, Mayo Clinic, Rochester, MN

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.


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