Abstract
The spectrum of cutaneous CD30-positive lymphoproliferative disorders encompasses both inflammatory and neoplastic conditions. CD30+ Hodgkin and Reed–Sternberg-like cells have been occasionally reported in primary cutaneous marginal zone lymphoma, where they are thought to represent a side neoplastic component within a dominant background of lymphomatous small B cells. Herein, we describe the histological and molecular findings of three cases of primary cutaneous marginal zone lymphomas with CD30+ H/RS cells, in which next-generation sequencing analysis revealed the clonal population to consist in less than 5% of the cutaneous B-cell infiltrate, providing a thought-provoking focus on a possible main role for CD30+ cells in primary cutaneous marginal zone lymphoproliferations.
Highlights
Primary cutaneous marginal zone lymphomas represent approximately 2–7% of all cutaneous lymphomas
The 2018 updated WHO-EORTC classification for primary cutaneous lymphomas recognizes two different subtypes of PCMZL based on the expression of class-switched immunoglobulins and the chemokine receptor CXCR3 [1,2,16,17]
A monoclonal rearrangement of immunoglobulin heavy (IGH) or light chain (IGK/IGL) genes can help in distinguishing PCMZL from cutaneous lymphoid hyperplasia (B-cell pseudolymphoma), which can histologically mimic PCMZL
Summary
Primary cutaneous marginal zone lymphomas represent approximately 2–7% of all cutaneous lymphomas. Follicles with reactive germinal centers may be present and colonized by the neoplastic marginal zone cells and plasma cells [2,11,12,13,14,15]. Due to the lack of colonization of reactive germinal centers by neoplastic B cells, lymphoepithelial lesions, or transformation into a diffuse large B-cell lymphoma, these cases are regarded by some authors as clonal chronic cutaneous lymphoproliferative disorders (LPD) rather than overt lymphomas [1,2,11,16,17,18,19,20]. The non-class-switched cases are instead considered true lymphomas consisting of large nodules of neoplastic B cells expressing IgM and CXCR3 with scattered plasma cells and a less prominent T-cell infiltrate. We describe three cases of PCMZL with CD30+ H/RS cells, which were investigated by using histopathology and genetic analysis of immunoglobulin gene rearrangement with sub-clonal resolution in an attempt to clarify the possible impact of the CD30-positive component
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