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Serum tryptase monitoring in indolent systemic mastocytosis: association with disease features and patient outcome

AutorMatito, Almudena; Morgado, J. M.; Álvarez-Twose, Iván; Sánchez-Muñoz, Laura; Pedreira, C. E.; Jara-Acevedo, Maria; Teodosio, Cristina; García-Montero, Andrés ; Orfao, Alberto ; Escribano, Luis
Fecha de publicación2013
EditorPublic Library of Science
CitaciónPLoS ONE 8(10): e76116 (2013)
Resumen[Background]: Serum baseline tryptase (sBT) is a minor diagnostic criterion for systemic mastocytosis (SM) of undetermined prognostic impact. We monitored sBT levels in indolent SM (ISM) patients and investigated its utility for predicting disease behaviour and outcome. [Methods]: In total 74 adult ISM patients who were followed for ≥48 months and received no cytoreductive therapy were retrospectively studied. Patients were classified according to the pattern of evolution of sBT observed. [Results]: Overall 16/74 (22%) cases had decreasing sBT levels, 48 (65%) patients showed increasing sBT levels and 10 (13%) patients showed a fluctuating pattern. Patients with significantly increasing sBT (sBT slope ≥0.15) after 48 months of follow-up showed a slightly greater rate of development of diffuse bone sclerosis (13% vs. 2%) and hepatomegaly plus splenomegaly (16% vs. 5%), as well as a significantly greater frequency of multilineage vs. mast cells (MC)-restricted KIT mutation (p = 0.01) together with a greater frequency of cases with progression of ISM to smouldering and aggressive SM (p = 0.03), and a shorter progression-free survival (p = 0.03). [Conclusions]: Monitoring of sBT in ISM patients is closely associated with poor prognosis disease features as well as with disease progression, pointing out the need for a closer follow-up in ISM patients with progressively increasing sBT values.
DescripciónThis is an open-access article distributed under the terms of the Creative Commons Attribution License.-- et al.
Versión del editorhttp://dx.doi.org/10.1371/journal.pone.0076116
URIhttp://hdl.handle.net/10261/134514
DOI10.1371/journal.pone.0076116
Identificadoresdoi: 10.1371/journal.pone.0076116
issn: 1932-6203
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