Abstract
Systemic mastocytosis (SM) is a rare form of mastocytosis characterized by a clonal proliferation of neoplastic mast cells in one or more extracutaneous organs. The infiltration of mast cells into the bone marrow leads to bone involvement, which is frequently the initial form of manifestation of the disease. In some cases, the bone lesions are the sole way of manifestation, making systemic mastocytosis difficult to diagnose. Fragility fractures, severe secondary osteoporosis and condensing or lytic bone lesions are the most common conditions, inducing a high burden of disease especially in younger population.
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