Splenomegaly, elevated alkaline phosphatase and mutations in the SRSF2/ASXL1/RUNX1 gene panel are strong adverse prognostic markers in patients with systemic mastocytosis

We evaluated the impact of clinical and molecular characteristics on overall survival (OS) in 108 patients with indolent (n=41) and advanced systemic mastocytosis (SM) (advSM, n=67). Organomegaly was measured by magnetic resonance imaging-based volumetry of the liver and spleen. In multivariate anal...

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Bibliographic Details
Published in:Leukemia 2016-12, Vol.30 (12), p.2342-2350
Main Authors: Jawhar, M, Schwaab, J, Hausmann, D, Clemens, J, Naumann, N, Henzler, T, Horny, H-P, Sotlar, K, Schoenberg, S O, Cross, N C P, Fabarius, A, Hofmann, W-K, Valent, P, Metzgeroth, G, Reiter, A
Format: Article
Language:English
Subjects:
Adult
Aged
Alkaline phosphatase
Alkaline Phosphatase - blood
Cancer
Care and treatment
Complications and side effects
Confidence intervals
Core Binding Factor Alpha 2 Subunit - genetics
Diagnosis
Female
Gene mutation
Gene mutations
Genetic aspects
Health aspects
Hematology
Humans
Kinases
Leukemia
Liver
Magnetic resonance imaging
Male
Markers
Mast cell disease
Mastocytosis
Mastocytosis, Systemic - diagnosis
Mastocytosis, Systemic - genetics
Mastocytosis, Systemic - mortality
Mastocytosis, Systemic - pathology
Medical prognosis
Middle Aged
Multivariate analysis
Mutation
Oncology
Oncology, Experimental
Parameters
Patients
Phosphatase
Prognosis
Repressor Proteins - genetics
Risk
Risk factors
Runx1 protein
Serine-Arginine Splicing Factors - genetics
Spleen
Splenomegaly
Splenomegaly - diagnostic imaging
Splenomegaly - pathology
Survival Rate
Volumetric analysis
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Summary:We evaluated the impact of clinical and molecular characteristics on overall survival (OS) in 108 patients with indolent (n=41) and advanced systemic mastocytosis (SM) (advSM, n=67). Organomegaly was measured by magnetic resonance imaging-based volumetry of the liver and spleen. In multivariate analysis of all patients, an increased spleen volume ⩾450 ml (hazard ratio (HR), 5.2; 95% confidence interval (CI), (2.1-13.0); P=0.003) and an elevated alkaline phosphatase (AP; HR 5.0 (1.1-22.2); P=0.02) were associated with adverse OS. The 3-year OS was 100, 77, and 39%, respectively (P
ISSN:0887-6924
1476-5551
DOI:10.1038/leu.2016.190