title: Prognostically relevant breakdown of 123 patients with systemic mastocytosis associated with other myeloid malignancies.
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.
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN 55905, USA. Pardanani.animesh@mayo.edu
authors.forename: Animesh
authors.initials: A
authors.lastname: Pardanani
authors.forename: Ken-Hong
authors.initials: KH
authors.lastname: Lim
authors.forename: Terra L
authors.initials: TL
authors.lastname: Lasho
authors.forename: Christy
authors.initials: C
authors.lastname: Finke
authors.forename: Rebecca F
authors.initials: RF
authors.lastname: McClure
authors.forename: Chin-Yang
authors.initials: CY
authors.lastname: Li
authors.forename: Ayalew
authors.initials: A
authors.lastname: Tefferi
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D004802:Eosinophilia / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics / Q000378:metabolism; D005260:Female; D019337:Hematologic Neoplasms / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology* / Q000235:genetics / Q000378:metabolism; D006801:Humans; D008297:Male; D034721:Mastocytosis, Systemic / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology* / Q000235:genetics / Q000378:metabolism; D008875:Middle Aged; D016609:Neoplasms, Second Primary / Q000175:diagnosis / Q000453:epidemiology* / Q000235:genetics / Q000378:metabolism; D015514:Oncogene Proteins, Fusion / Q000235:genetics / Q000378:metabolism; D011379:Prognosis; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000235:genetics / Q000378:metabolism; D039221:mRNA Cleavage and Polyadenylation Factors / Q000235:genetics / Q000378:metabolism
chemical_list: D015514:Oncogene Proteins, Fusion; D039221:mRNA Cleavage and Polyadenylation Factors; C479819:FIP1L1-PDGFRA fusion protein, human; D020796:Receptor, Platelet-Derived Growth Factor alpha