TY - JOUR
T1 - Prognostically relevant breakdown of 123 patients with systemic mastocytosis associated with other myeloid malignancies
AU - Pardanani, Animesh
AU - Lim, Ken Hong
AU - Lasho, Terra L.
AU - Finke, Christy
AU - McClure, Rebecca F.
AU - Li, Chin Yang
AU - Tefferi, Ayalew
PY - 2009/10/29
Y1 - 2009/10/29
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=70449706224&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=70449706224&partnerID=8YFLogxK
U2 - 10.1182/blood-2009-05-220145
DO - 10.1182/blood-2009-05-220145
M3 - Article
C2 - 19713463
AN - SCOPUS:70449706224
SN - 0006-4971
VL - 114
SP - 3769
EP - 3772
JO - Blood
JF - Blood
IS - 18
ER -