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Case Report



Aggressive systemic mastocytosis: a silent enemy

Joana Alves Vaz, Maria Alice Sousa, Celina Afonso, Rita Sampaio.


Abstract

Mastocytosis results from the accumulation, proliferation and activation of mast cells with the involvement of several organs. The skin, bone marrow, gastrointestinal tract, liver, spleen and lymph nodes are the most frequent locations. Symptoms result from the accumulation of mast cells and the release of mediators from their activation. The incidence of this pathology is rare and, depending on the presentation, the diagnosis can be difficult. Systemic presentation is more frequent in adults, in contrast to the pediatric population, where skin disease prevails and with a good prognosis.
The authors describe the case of a man diagnosed with chronic anaemia and being followed up in a Hematology consultation who presents with anorexia, asthenia and weight loss. After inpatient assessment, the diagnosis of aggressive systemic mastocytosis was established.

Key words: Aggressive systemic mastocytosis, chronic anaemia






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