• Geisilene Russano de Paiva Silva, Emilie Tournier, Luis Otávio Sarian, Cristina Bulai-Livideanu, Georges Delsol, Laurence Lamant, José Vassallo, Pierre Brousset, and Camille Laurent.
• UMR U.1037, Centre de recherche sur cancer de Toulouse, Université Paul-Sabatier Department of Pathology, Institut Universitaire du Cancer de Toulouse-Oncopole Department of Dermatology, Paul Sabatier University, Mastocytosis National Reference Center (CEREMAST), Toulouse University and CHU, Toulouse, France Laboratory of Molecular and Investigative Pathology-LAPE, Faculty of Medical Sciences, State University of Campinas Medical School, Campinas, Brazil Laboratoire d'excellence Labex TOUCAN, Toulouse, France.
• Medicine (Baltimore). 2018 May 1; 97 (21): e10642e10642.

AbstractMastocytosis is a rare disease characterized by clonal neoplastic proliferation of mast cells (MCs). It ranges from skin lesions as cutaneous mastocytosis (CM) which may spontaneously regress to highly aggressive neoplasms with multiorgan involvement corresponding to some aggressive systemic mastocytosis (ASM), mast cell leukemia (MCL), and/or mast cell sarcoma (MCS).There is increasing evidence of CD30 expression in neoplastic MCs of the bone marrow. This expression has been described almost exclusively in aggressive forms of systemic mastocytosis (SM).The aim of the present study is to evaluate CD30 expression both in cutaneous and systemic forms of mastocytosis. Forty-two mastocytosis cases were reviewed, including cutaneous (n = 29) and systemic (n = 13) forms to assess the prevalence of CD30 expression. Thirty-nine out of 42 (92.8%) cases were CD30 positive. In cases of CM, 28/29 (96.5%) cases were CD30 positive, 11/13 cases of SM (84.6%) were positive for CD30. MCs in normal skin biopsies and in urticaria lesions were CD30-negative. This study found that CD30 is also frequently expressed in CM as well as in systemic forms. This finding is a major departure from the prevailing concept that CD30 expression is often related to aggressive systemic forms of mastocytosis.

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