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Large Cells With CD30 Expression and Hodgkin-like Features in Primary Cutaneous Marginal Zone B-Cell Lymphoma

A Study of 13 Cases

Prieto-Torres, Lucía MD*; Manso, Rebeca PhD; Cieza-Díaz, Deysy Elisabeth PhD*; Jo, Margarita MD; Kilany Pérez, Linah; Montenegro-Damaso, Társila MD; Eraña, Itziar MD; Lorda, Marta PhD§; Suarez Massa, Dolores PhD; Machan, Salma MD*; Córdoba, Raúl PhD; Ara, Mariano PhD§; Requena, Luis PhD*; Rodriguez-Pinilla, Socorro M. PhD†,#; Piris, Miguel A. PhD†,#

The American Journal of Surgical Pathology: September 2019 - Volume 43 - Issue 9 - p 1191–1202
doi: 10.1097/PAS.0000000000001287
Original Articles
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The presence of CD30+ cells in cutaneous lymphomas has come to prominence in recent years as a potential diagnostic and therapeutic marker. In primary cutaneous marginal zone B-cell lymphomas, the presence of large CD30+ cells with Hodgkin-like features and their significance have not yet been studied. Here we describe the main clinical, histologic, immunophenotypic, and molecular characteristics of 13 cases of primary cutaneous marginal zone lymphomas featuring >10% of CD30+ large cells, and analyze their relationship with histologic and clinical progression of the disease and with other morphologic and immunophenotypic features. We report 10 male and 3 female patients, 4 with early-local disease and 8 with locoregional advanced disease without extracutaneous involvement but with a high relapse rate of 69%. We describe an association between a high level of CD30 expression and disease progression, with increased clinical recurrence in cases with >15% of CD30+ cells. We also discuss the differential diagnosis with other cutaneous and systemic lymphomas, especially Hodgkin lymphoma.

Departments of *Dermatology

Pathology

Hematology of the Fundación Jiménez Díaz University Hospital

#CIBERONC, Madrid

Department of Pathology, Insular maternal-child university hospital complex, Canaries

§Department of Dermatology, Lozano Blesa Clinical University Hospital, Zaragoza

Department of Pathology, Puerta de Hierro University Hospital, Majadahonda, Spain

Conflicts of Interest and Source of Funding: This work was supported by grants from the Instituto de Salud Carlos III (ISCIII) of the Spanish Ministry of Economy and Competence (MINECO, RTICC ISCIII and CIBERONC) (SAF2013-47416-R, RD06/0020/0107-RD012/0036/0060 and Plan Nacional I+D+I: PI17/2172, PI16/01294 and PIE15/0081), AECC, and the Madrid Autonomous Community. M.A.P. declares having received lecture fees and advisory board fees from Takeda, Janssen, and Celgene. The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article.

Correspondence: Lucía Prieto-Torres, MD, Department of Dermatology, Hospital Universitario, Fundación Jiménez Díaz, Madrid 28040, Spain (e-mail: lucia14_prie@msn.com).

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