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

• Published in: Blood 2009-10, Vol.114 (18), p.3769-3772
• Main Authors: Pardanani, Animesh, Lim, Ken-Hong, Lasho, Terra L., Finke, Christy, McClure, Rebecca F., Li, Chin-Yang, Tefferi, Ayalew
• Format: Article
• Language: English
• Subjects: Adult; Aged; Aged, 80 and over; Biological and medical sciences; Eosinophilia - complications; Eosinophilia - diagnosis; Eosinophilia - epidemiology; Eosinophilia - genetics; Eosinophilia - metabolism; Female; Hematologic and hematopoietic diseases; Hematologic Neoplasms - complications; Hematologic Neoplasms - diagnosis; Hematologic Neoplasms - epidemiology; Hematologic Neoplasms - genetics; Hematologic Neoplasms - metabolism; Humans; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Male; Mastocytosis, Systemic - complications; Mastocytosis, Systemic - diagnosis; Mastocytosis, Systemic - epidemiology; Mastocytosis, Systemic - genetics; Mastocytosis, Systemic - metabolism; Medical sciences; Middle Aged; mRNA Cleavage and Polyadenylation Factors - genetics; mRNA Cleavage and Polyadenylation Factors - metabolism; Neoplasms, Second Primary - diagnosis; Neoplasms, Second Primary - epidemiology; Neoplasms, Second Primary - genetics; Neoplasms, Second Primary - metabolism; Oncogene Proteins, Fusion - genetics; Oncogene Proteins, Fusion - metabolism; Prognosis; Receptor, Platelet-Derived Growth Factor alpha - genetics; Receptor, Platelet-Derived Growth Factor alpha - metabolism
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood-2009-05-220145

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.