Cutaneous T‐cell lymphomas: 2021 update on diagnosis, risk‐stratification, and management

Abstract

Disease overview: Cutaneous T-cell lymphomas are a heterogenous group of T-cell neoplasms involving the skin, the majority of which may be classified as Mycosis Fungoides (MF) or Sézary Syndrome (SS). Diagnosis: The diagnosis of MF or SS requires the integration of clinical and histopathologic data.Risk-adapted therapy: TNMB (tumor, node, metastasis, blood) staging remains the most important prognostic factor in MF/SS and forms the basis for a "risk-adapted," multi-disciplinary approach to treatment. For patients with disease limited to the skin, expectant management or skin-directed therapies is preferred, as both diseasespecific and overall survival for these patients is favorable. In contrast, patients with advanced-stage disease with significant nodal, visceral or blood involvement are generally approached with systemic therapies, including biologic-response modifiers, histone deacetylase inhibitors, or antibody-based strategies, in an escalating fashion. In highly-selected patients, allogeneic stem-cell transplantation may be considered, as this may be curative in some patients.

Brentuximab extravasation injury in a patient with mycosis fungoides with large cell transformation

“…Primary cutaneous lymphomas are non‐Hodgkin lymphomas without extracutaneous disease at the time of diagnosis and are typically indolent. 1 , 2 CD30 positivity may suggest the potential for large cell transformation and progression. 3 Brentuximab vedotin (BV) is an anti‐CD30 antibody‐drug conjugate newly approved to treat CD30+ cutaneous lymphomas.…”

Epstein–Barr virus‐associated leiomyosarcoma of the larynx in an adult patient with human immunodeficiency virus infection: Case report and review of the literature

“…113 Patients with Sézary syndrome (SS) are predisposed to secondary malignancies. 115,116 SS is a leukemic form of epidermotropic cutaneous T-cell lymphoma characterized by pruritus and squamous erythroderma. Among patients with SS, 27% develop a secondary malignancy.…”

Malignant and Benign T Cells Constituting Cutaneous T-Cell Lymphoma

“…Antigen-specific T cells overexpressing CD5 reportedly persist better as memory T cells after peripheral activation [ 93 ]. The loss of CD5 is sometimes seen in the advanced stages of MF [ 94 ]. However, the expression of CD5 is also reported to be higher in the malignant T cells of SS compared to the benign T cells [ 89 ].…”

“…CTCL is treated with various modalities, including topical therapies, ultraviolet (UV) therapies, radiation therapies, and systemic therapies [ 94 ]. It has been reported that the cytokine profile of benign T cells is significantly changed before and after effective treatments.…”