Abstract

RAMA, Tiago Azenha; MOREIRA, André  and  DELGADO, Luís. Diagnostic and treatment approaches to mastocytosis - A proposal for clinical guidelines. Rev Port Imunoalergologia [online]. 2020, vol.28, n.1, pp.31-49. ISSN 0871-9721.  https://doi.org/10.32932/rpia.2020.03.030.

Mastocytosis are a heterogeneous group of rare clonal mast cell diseases. In the past, mastocytosis management included a strict avoidance of all drugs, contrast media, foods and procedures ever described as mast cell activators. In the present, several international guidelines have emerged, proposing a more tailored approach, however in Portugal there are no current clinical guidelines. This proposal for management of mastocytosis intends to offer guidelines to the Portuguese clinician. Diagnosis should be based on a skin biopsy (when there are skin lesions) and on bone marrow examination. All patients should have documentation on their disease and triggers that should be avoided. Treatment should be personalized and may include cromolyn sodium, H1 and/or H2 antihistamines, among others. Patients should be given premedication protocols to be implemented before surgical procedures, labour and use of iodinated contrast media.

Keywords : Mastocytosis; mast cells; cutaneous mastocytosis; systemic mastocytosis.

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