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.
Pardanani
Animesh
A
Division of Hematology, Mayo Clinic, Rochester, MN 55905, USA. Pardanani.animesh@mayo.edu
Lim
Ken-Hong
KH
Lasho
Terra L
TL
Finke
Christy
C
McClure
Rebecca F
RF
Li
Chin-Yang
CY
Tefferi
Ayalew
A
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
D015514:Oncogene Proteins, Fusion; D039221:mRNA Cleavage and Polyadenylation Factors; C479819:FIP1L1-PDGFRA fusion protein, human; D020796:Receptor, Platelet-Derived Growth Factor alpha