Association of systemic mastocytosis with primary cutaneous marginal zone lymphoma; first case.

Mastocytosis is the result of a clonal, neoplastic proliferation of mast cells that accumulate in one or more organ systems and has two main types; cutaneous and systemic mastocytosis (SM). Systemic type is classified as indolent SM, smouldering SM, SM with an associated hematological neoplasm (SM-AHN), aggressive SM, mast cell leukemia and mast cell sarcoma¹. As relationship of SM and hematologic disorders is well known, its association with primary cutaneous lymphomas (PCLs) is very rare²⁻⁴.