Russano de Paiva Silva Geisilene, Tournier Emilie, Sarian Luis Otávio, Bulai-Livideanu Cristina, Delsol Georges, Lamant Laurence, Vassallo José, Brousset Pierre, Laurent Camille
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;97(21):e10642. doi: 10.1097/MD.0000000000010642.
Mastocytosis 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.
肥大细胞增多症是一种罕见疾病，其特征为肥大细胞（MC）的克隆性肿瘤增殖。它涵盖了从可能自发消退的皮肤病变如皮肤肥大细胞增多症（CM）到多器官受累的高度侵袭性肿瘤，后者对应于某些侵袭性系统性肥大细胞增多症（ASM）、肥大细胞白血病（MCL）和/或肥大细胞肉瘤（MCS）。越来越多的证据表明骨髓肿瘤性MC中存在CD30表达。这种表达几乎仅在侵袭性系统性肥大细胞增多症（SM）形式中被描述。本研究的目的是评估肥大细胞增多症皮肤型和系统型中CD30的表达情况。回顾了42例肥大细胞增多症病例，包括皮肤型（n = 29）和系统型（n = 13），以评估CD30表达的发生率。42例中有39例（92.8%）为CD30阳性。在CM病例中，28/29（96.5%）例为CD30阳性，11/13例SM（84.6%）为CD30阳性。正常皮肤活检和荨麻疹病变中的MC为CD30阴性。本研究发现CD30在CM以及系统型中也经常表达。这一发现与普遍认为CD30表达常与侵袭性系统性肥大细胞增多症形式相关的观念大相径庭。
