Advances in the understanding and clinical management of mastocytosis and clonal mast cell activation syndromes [version 1; referees: 2 approved]
David González-de-Olano,
Almudena Matito,
Alberto Orfao,
Luis Escribano
Affiliations
David González-de-Olano
Department of Allergy, Hospital Universitario Ramón y Cajal, Madrid, 28034, Spain
Almudena Matito
Instituto de Estudios de Mastocitosis de Castilla La Mancha (CLMast), Hospital Virgen del Valle, Toledo, 45071, Spain
Alberto Orfao
Centro de Investigación del Cáncer/IBMCC (CSIC/USAL), Departamento de Medicina, IBSAL and Servicio General de Citometría, University of Salamanca, Salamanca, 37007, Spain
Luis Escribano
Centro de Investigación del Cáncer/IBMCC (CSIC/USAL), Departamento de Medicina, IBSAL and Servicio General de Citometría, University of Salamanca, Salamanca, 37007, Spain
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.