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F1000Res. 2016 Nov 14;5:2666. doi: 10.12688/f1000research.9565.1. eCollection 2016.

Advances in the understanding and clinical management of mastocytosis and clonal mast cell activation syndromes.

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Department of Allergy, Hospital Universitario Ramón y Cajal, Madrid, 28034, Spain.
Instituto de Estudios de Mastocitosis de Castilla La Mancha (CLMast), Hospital Virgen del Valle, Toledo, 45071, Spain.
Centro de Investigación del Cáncer/IBMCC (CSIC/USAL), Departamento de Medicina, IBSAL and Servicio General de Citometría, University of Salamanca, Salamanca, 37007, Spain.
Contributed equally


Clonal mast cell activation syndromes and indolent systemic mastocytosis without skin involvement are two emerging entities that sometimes might be clinically difficult to distinguish, and they involve a great challenge for the physician from both a diagnostic and a therapeutic point of view. Furthermore, final diagnosis of both entities requires a bone marrow study; it is recommended that this be done in reference centers. In this article, we address the current consensus and guidelines for the suspicion, diagnosis, classification, treatment, and management of these two entities.


Management; Mast Cell Activation Syndromes; Mastocytosis; Treatment

Conflict of interest statement

The authors declare that they have no competing interests. No competing interests were disclosed. No competing interests were disclosed.

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