Cutaneous T‐cell lymphomas: 2023 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.

Mechanism of action of chlormethine gel in mycosis fungoides

“…6,12 The discontinuous nature of disease progression in MF is characterized by variable rates of tumour progression, from rapid (over a period of weeks) to being stable for protracted periods. 20 Numerous treatments and established guidelines are available, 21 but the relative scarcity of randomized, comparative studies in this area has led to heterogeneous treatment approaches 3,18,22,23 and hindered the development of clear, universally applicable treatment algorithms. 18,22,23 Hence, a 'one-size-fits-all' approach is not currently possible.…”

Adolescent with primary cutaneous follicle center lymphoma treated with rituximab

“…LETTER TO THE EDITOR Primary cutaneous B-cell lymphomas are extra-nodal NHLs that include the subtypes such as primary cutaneous marginal zone B-cell lymphoma, PCFCL, primary cutaneous diffuse large B-cell lymphoma, leg type and Epstein-Barr virus (EBV)-positive mucocutaneous ulcers. [1][2][3] Patients with PCFCL are typically older males with cutaneous disease. PCFCL is very rarely seen in children.…”

“…PCFCL is very rarely seen in children. [3][4][5][6][7][8] PCFCL typically presents as a solitary lesion or several erythematous papules or nodules on the head, neck, and trunk without systemic symptoms or disseminated disease. 3 Survival rates are excellent, greater than 95%, although survival is lower if lesions are present on the lower extremities.…”

“…[3][4][5][6][7][8] PCFCL typically presents as a solitary lesion or several erythematous papules or nodules on the head, neck, and trunk without systemic symptoms or disseminated disease. 3 Survival rates are excellent, greater than 95%, although survival is lower if lesions are present on the lower extremities. 3,9 The differential diagnosis for these lesions may include follicular lymphoma, diffuse large B-cell lymphoma leg type, reactive lymphoid hyperplasia, and primary cutaneous marginal zone lymphoma.…”

“…3 Survival rates are excellent, greater than 95%, although survival is lower if lesions are present on the lower extremities. 3,9 The differential diagnosis for these lesions may include follicular lymphoma, diffuse large B-cell lymphoma leg type, reactive lymphoid hyperplasia, and primary cutaneous marginal zone lymphoma. These entities must be differentiated pathologically after biopsy is obtained.…”