Background: Mastocytosis includes a range of disorders characterised by accumulation of tissue mast cells. These are derived from pluripotent haematopoietic stem cells. Recent research has improved the understanding of the mastocytosis pathogenesis. Organ manifestations and symptoms are highly variable.
Material and methods: Two cases of systemic mast cell disease are presented.
Results: One patient had urticaria pigmentosa and systemic mast cell disease; the other had systemic mast cell disease and myelodysplastic changes in the bone marrow.
Interpretation: The two cases illustrate different manifestations and different prognosis for various types of mastocytosis.