Background: Previous investigations pointed out a role for antigen stimulation in Sezary syndrome (SS). High-throughput sequencing of the T cell receptor (TR) offers several applications beyond diagnostic purposes, including the study of T cell pathogenesis.
Methods: We performed high-throughput RNA sequencing of the TR alpha (TRA) and beta (TRB) genes focusing on the complementarity-determining region 3 (CDR3) in 11 SS and one erythrodermic mycosis fungoides (MF) patients.
Background: Brentuximab vedotin (BV) has been approved for CD30-expressing cutaneous T-cell lymphoma (CTCL) after at least one previous systemic treatment. However, real clinical practice is still limited.
Objectives: To evaluate the response and tolerance of BV in a cohort of patients with CTCL.
Advanced cutaneous T cell lymphoma (CTCL) patients have a dismal prognosis, especially those relapsing or progressing after systemic therapy. No curative therapies are available, but some new agents have prolonged disease-free survival time with a good toxicity profile. Allogeneic stem cell transplantation (allo-SCT) offers the longest disease-free survival, potentially representing the best therapeutic option for eligible patients.
View Article and Find Full Text PDFWithin T-cell lymphomas (TCL) there are 2 entities expressing gamma-delta TCR: hepatosplenic gamma-delta T-cell lymphoma (HSGDTL) and the primary cutaneous gamma-delta T-cell lymphoma (PCGDTL). PCGDTL is a rare form of Tcell lymphoma with specific tropism for skin that have a dismal prognosis. Although even rarer, there have been reports of TCL with loss of expression of the TCR, which have been termed peripheral TCL TCR-silent type.
View Article and Find Full Text PDFThis study evaluated the efficacy and tolerability of topical timolol, 0.5%, gel as a treatment of paronychia and pseudopyogenic granuloma induced by antineoplastic agents.
View Article and Find Full Text PDFMycosis fungoides and Sézary syndrome (MF/SS) are the most common forms of primary cutaneous T cell lymphomas. We analyzed the applicability of the cutaneous lymphoma international prognostic index (CLIPi) in MF/SS. We introduced the total body-surface area affected (TBSA) and the type of skin lesions at diagnosis as prognostic variables.
View Article and Find Full Text PDFJ Dermatolog Treat
September 2016
Introduction: Sézary syndrome (SS) is characterized by rapidly progressive disease and poor survival. Although there is no standard treatment for SS, allogeneic stem cell transplantation (alloSCT) is the only treatment available that may offer a long survival. Alemtuzumab, a humanized monoclonal antibody that targets CD52, has reported some efficacy in this disease.
View Article and Find Full Text PDFBackground: Primary cutaneous marginal zone B-cell lymphomas are low-grade lymphomas running an indolent course. Skin relapses have been frequently reported but little information about disease-free survival (DFS) is available.
Objective: We sought to evaluate relapse rate and DFS in patients with primary cutaneous marginal zone B-cell lymphomas.
Background: Primary cutaneous diffuse large B-cell lymphoma (PCDLBCL), leg type can eventually disseminate to extracutaneous sites including testes. In addition, patients with testicular lymphoma can develop specific skin involvement.
Objective: We sought to describe similarities between PCDLBCL, leg type and testicular B-cell lymphoma affecting the skin.
Background: Folliculotropic mycosis fungoides (MF) is a rare variant of cutaneous T-cell lymphoma in which the neoplastic T lymphocytes display tropism for the follicular epithelium.
Objectives: To better categorize this rare form of cutaneous T-cell lymphoma we evaluated the clinical, pathological, and immunophenotypic findings, and the response to therapy and course of the disease.
Methods: Folliculotropic MF cases were selected from the registry of the Thematic Network of Cutaneous Lymphoma of Barcelona (Spain) from 1988 to 2007.
Four patients presenting a peculiar clinical variant of erythema annulare centrifugum are reported. The lesions were clinically and histopathologically indistinguishable from classic superficial erythema annulare centrifugum but constant annual and seasonal recurrences for many years or decades were observed. No clear precipitating factor could be identified.
View Article and Find Full Text PDFEpithelioid granuloma formation has rarely been observed in specific cutaneous lesions from T-cell lymphomas other than those of mycosis fungoides/Sézary syndrome (MF/SS). Three patients diagnosed with nodal and/or extranodal (tonsillar) non-Hodgkin's peripheral T-cell lymphoma (PTCL) and one patient with angioimmunoblastic T-cell lymphoma (AILD), developed specific cutaneous involvement showing prominent epithelioid cell and/or granulomatous inflammation. The original diagnostic lesions had no granulomatous features.
View Article and Find Full Text PDFThe aim of the present study was to identify genetic aberrations in a series of patients with cutaneous large B-cell lymphoma (LBCL) using comparative genomic hybridization (CGH). Eighteen consecutive patients with primary (13 patients) (PCLBCL) and secondary (five patients) (SCLBCL) cutaneous large B-cell lymphoma were included in the study. Nine cases corresponded to PCLBCL leg type and four cases primary cutaneous follicle centre-cell lymphoma (PCFCL).
View Article and Find Full Text PDFBackground And Objectives: Sezary's syndrome is a peripheral T-cell neoplasm characterized by a pruritic exfoliative or infiltrated erythroderma, lymphadenopathies, and atypical T lymphocytes in the peripheral blood. Cytogenetic studies are scarce. This study was designed to increase cytogenetic information on this disorder.
View Article and Find Full Text PDF