Un caso di linfoadenopatia, epatosplenomegalia e triptasi elevata

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ABSTRACT

A case of lymphadenopathy, hepatosplenomegaly and elevated serum tryptase level

A case of lymphadenopathy, hepatosplenomegaly and elevated serum tryptase level. Mastocytosis is a rare clonal disease characterized by neoplastic proliferation of mast cells in one or more organs, frequently skin and bone marrow. Diagnosis and classification of mastocytosis is based on the identification of neoplastic mast cells in according to the morphological, immunophenotypical and/or molecular criteria established by the WHO. For the diagnosis, serum levels of the tryptase which correlate with quantity and activity of mast cells is particulary useful. This case reports about a 60-year-old man with hepatosplenomegaly, modest monoclonal component, reactive lymph node hyperplasia and moderate macrocytic anemia (haemoglobin:129 gr/L). Bone marrow aspiration shows areas of infiltration of atypical mast cells type II and rarely type I with aberrant immunophenotype: CD45++CD117++CD2+CD25+. The tryptase concentration is significantly increased. According to WHO 2008 criteria, diagnosis of systemic mastocytosis is made. Early diagnosis of mastocytosis is pivotal because immunotherapy is often required to reduce the risk of allergic reactions or major bone complications.

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