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1.
Eur J Cancer ; 133: 120-130, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32502876

RESUMO

BACKGROUND: Brentuximab vedotin was approved for adult patients with CD30-expressing cutaneous T-cell lymphoma treated with prior systemic therapy based on improved response rates and progression-free survival with brentuximab vedotin (1.8 mg/kg once every 3 weeks; ≤16 cycles) versus physician's choice (methotrexate/bexarotene; ≤48 weeks) in the phase III ALCANZA study. Quality of life (QoL) in ALCANZA patients was also examined. METHODS: QoL measures in ALCANZA were based on the Skindex-29, Functional Assessment of Cancer Therapy-General (FACT-G) and European QoL 5-dimension (EQ-5D) questionnaires. RESULTS: Mean maximum reduction from the baseline Skindex-29 symptom domain score (key secondary end-point) was greater with brentuximab vedotin than physician's choice (-27.96 versus -8.62); the difference, -18.9 (95% confidence interval -26.6, -11.2; adjusted p < 0.001), exceeded the study-defined minimally important difference (9.0-12.3). Mean changes from baseline to end-of-treatment visit total FACT-G scores were similar with brentuximab vedotin and physician's choice (0.15 versus -2.29). EQ-5D changes were also comparable between arms. Among brentuximab vedotin-treated patients with peripheral neuropathy (PN), mean maximum reduction in Skindex-29 symptom domain was -35.54 versus -11.11 in patients without PN. PN had no meaningful effect on FACT-G and EQ-5D QoL scores. CONCLUSIONS: In summary, brentuximab vedotin produced superior reductions in symptom burden compared with physician's choice, without adversely impacting QoL. QoL was unaffected by the presence of PN in brentuximab vedotin-treated patients. CLINICAL TRIAL REGISTRATION: NCT01578499.


Assuntos
Brentuximab Vedotin/uso terapêutico , Linfoma Cutâneo de Células T/tratamento farmacológico , Qualidade de Vida , Neoplasias Cutâneas/tratamento farmacológico , Adulto , Idoso , Idoso de 80 Anos ou mais , Resistencia a Medicamentos Antineoplásicos/efeitos dos fármacos , Feminino , Humanos , Linfoma Cutâneo de Células T/epidemiologia , Linfoma Cutâneo de Células T/patologia , Linfoma Cutâneo de Células T/psicologia , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/tratamento farmacológico , Recidiva Local de Neoplasia/epidemiologia , Recidiva Local de Neoplasia/psicologia , Medidas de Resultados Relatados pelo Paciente , Psicometria/métodos , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/psicologia , Inquéritos e Questionários , Resultado do Tratamento , Adulto Jovem
2.
Am J Surg Pathol ; 44(7): 862-872, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32271188

RESUMO

Primary cutaneous CD4 small/medium T-cell lymphoproliferative disorder (PCSMLPD) is a recently recognized entity in the 2017 World Health Organization (WHO) classification. It belongs to the T-follicular helper (TFH) lymphoproliferations. The clinical, pathologic, and molecular features of this localized disease are underresearched. We conducted a retrospective multicentric study of 60 patients with a PCSMLPD that presented as a single cutaneous lesion. Clinical, pathologic, and targeted molecular analyses were performed. PCSMLPD presented mostly as a nodule (45%), located on the head and neck area (50%) in adults (mean age: 59 y [43.3 to 75.2]). All patients had an indolent disease course, either at initial staging or during follow-up (mean: 16.6 mo [1.3 to 31.9]). Spontaneous regression was reported in 31.9% of cases. The infiltrates were most often nodular and/or diffuse, expanding in the whole dermis (78%, Pattern 1), rather than subepidermal band-like in the superficial dermis (22%, Pattern 2). Epidermotropism, folliculotropism, and capillary hyperplasia were common. The expression of TFH lineage markers was more extensive in lesions with Pattern 2, but a substantial B-cell infiltrate was seen in both types of lesions. A clonal rearrangement of the TCR genes was identified in 68% of cases. One sample of the 13 tested revealed a mutation in the DNMT3A gene among the 9 genes studied (TET2, DNMT3A, IDH2, RHOA, SETD2, PLCG1, STAT3, STAT5B, and CD28). PCSMLPD follows a benign clinical course and can spontaneously regress after biopsy. Although PCSMLPD expresses TFH lineage markers, mutations usually found in angioimmunoblastic T-cell lymphomas are uncommon.


Assuntos
Linfoma Cutâneo de Células T/diagnóstico , Neoplasias Cutâneas/diagnóstico , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores Tumorais/genética , Biomarcadores Tumorais/metabolismo , Linfócitos T CD4-Positivos/metabolismo , Feminino , Seguimentos , França , Humanos , Imuno-Histoquímica , Linfoma Cutâneo de Células T/genética , Linfoma Cutâneo de Células T/metabolismo , Linfoma Cutâneo de Células T/patologia , Masculino , Pessoa de Meia-Idade , Mutação , Fenótipo , Remissão Espontânea , Estudos Retrospectivos , Análise de Sequência de DNA , Neoplasias Cutâneas/genética , Neoplasias Cutâneas/metabolismo , Neoplasias Cutâneas/patologia
3.
Nat Commun ; 11(1): 1806, 2020 04 14.
Artigo em Inglês | MEDLINE | ID: mdl-32286303

RESUMO

Primary cutaneous γδ T cell lymphomas (PCGDTLs) represent a heterogeneous group of uncommon but aggressive cancers. Herein, we perform genome-wide DNA, RNA, and T cell receptor (TCR) sequencing on 29 cutaneous γδ lymphomas. We find that PCGDTLs are not uniformly derived from Vδ2 cells. Instead, the cell-of-origin depends on the tissue compartment from which the lymphomas are derived. Lymphomas arising from the outer layer of skin are derived from Vδ1 cells, the predominant γδ cell in the epidermis and dermis. In contrast, panniculitic lymphomas arise from Vδ2 cells, the predominant γδ T cell in the fat. We also show that TCR chain usage is non-random, suggesting common antigens for Vδ1 and Vδ2 lymphomas respectively. In addition, Vδ1 and Vδ2 PCGDTLs harbor similar genomic landscapes with potentially targetable oncogenic mutations in the JAK/STAT, MAPK, MYC, and chromatin modification pathways. Collectively, these findings suggest a paradigm for classifying, staging, and treating these diseases.


Assuntos
Linfoma Cutâneo de Células T/genética , Linfoma Cutâneo de Células T/patologia , Receptores de Antígenos de Linfócitos T gama-delta/metabolismo , Neoplasias Cutâneas/genética , Neoplasias Cutâneas/patologia , Sequência de Aminoácidos , Antígenos CD1d/metabolismo , Montagem e Desmontagem da Cromatina , Epitopos/imunologia , Genoma Humano , Células HEK293 , Humanos , Linfonodos/patologia , Modelos Biológicos , Mutação/genética , Fenótipo , Análise de Componente Principal , Transdução de Sinais , Pele/patologia , Transcrição Genética , Transcriptoma/genética
4.
Sci Rep ; 10(1): 2511, 2020 02 13.
Artigo em Inglês | MEDLINE | ID: mdl-32054948

RESUMO

MiR-34a and miR-16 coordinately control cell cycle checkpoint in non-small cell lung cancer (NSCLC) cells. In cutaneous T-cell lymphoma (CTCL) cells miR-16 regulates a switch between apoptosis and senescence, however the role of miR-34a in this process is unclear. Both miRNAs share many common targets and experimental evidences suggest that they synergistically control the cell-fate regulation of NSCLC. In this work we investigate whether the coordinate action between miR-34a and miR-16 can explain experimental results in multiple cell lines of NSCLC and CTCL. For that we propose a Boolean model of the G1/S checkpoint regulation contemplating the regulatory influences of both miRNAs. Model validation was performed by comparisons with experimental information from the following cell lines: A549, H460, H1299, MyLa and MJ presenting excellent agreement. The model integrates in a single logical framework the mechanisms responsible for cell fate decision in NSCLC and CTCL cells. From the model analysis we suggest that miR-34a is the main controller of miR-16 activity in these cells. The model also allows to investigate perturbations of single or more molecules with the purpose to intervene in cell fate mechanisms of NSCLC and CTCL cells.


Assuntos
Carcinoma Pulmonar de Células não Pequenas/genética , Neoplasias Pulmonares/genética , Linfoma Cutâneo de Células T/genética , MicroRNAs/genética , Neoplasias Cutâneas/genética , Carcinoma Pulmonar de Células não Pequenas/patologia , Pontos de Checagem do Ciclo Celular , Linhagem Celular Tumoral , Simulação por Computador , Regulação Neoplásica da Expressão Gênica , Humanos , Neoplasias Pulmonares/patologia , Linfoma Cutâneo de Células T/patologia , Modelos Biológicos , Neoplasias Cutâneas/patologia
5.
PLoS One ; 15(1): e0228046, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31978091

RESUMO

BACKGROUND: While mycosis fungoides (MF) and Sézary syndrome (SS) are the most common cutaneous lymphomas (CLs), there is limited data about non-MF/SS CLs. OBJECTIVE: We aimed to evaluate clinical characteristics of non-MF/SS CLs. METHODS: A retrospective analysis evaluated patients with non-MF/SS CLs covering a period of 17 years. The records of 59 patients with non-MF/SS CLs were reviewed for demographic profiles, clinical features, and survival outcomes. RESULTS: Our series consisted of 38 non-MF/SS cutaneous T-cell lymphomas (CTCLs) and 21 cutaneous B-cell lymphomas (CBCLs). In the group of non-MF/SS CTCLs including 33 primary and five secondary cases, there were cases of anaplastic large cell lymphoma (15.3% of non-MF/SS CLs), extranodal natural killer/ T-cell lymphoma (13.5%), peripheral T-cell lymphoma, not otherwise specified (13,5%), adult T-cell leukemia/lymphoma (8.5%), subcutaneous panniculitis-like T-cell lymphoma (6.8%) and angioimmunoblastic T-cell lymphoma (6.8%). In the group of CBCLs including nine primary and 12 secondary cases, there were cases of diffuse large B-cell lymphoma (22.0%), mantle cell lymphoma (5.1%), extranodal marginal lymphoma of mucosa associated lymphoid tissue (3.4%), follicle center lymphoma (3.4%) and intravascular large B-cell lymphoma (1.7%). The overall survivals were 57 months for non-MF/SS CTCLs and 41.5 months for CBCLs. Elevated serum lactate dehydrogenase level, thrombocytopenia, multiple anatomical sites of skin involvement and lower albumin level may be associated with poor prognosis in non-MF/SS CTCLs, but the latter two were not in CBCLs. CONCLUSION: With this series, we hope to provide indigenous data and outcome of non-MF/SS CLs. The overall survival of non-MF/SS CTCLs was better than CBCLs.


Assuntos
Linfoma Cutâneo de Células T/patologia , Encaminhamento e Consulta , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Micose Fungoide/patologia , Estadiamento de Neoplasias , Prognóstico , Estudos Retrospectivos , Síndrome de Sézary/patologia , Análise de Sobrevida , Taiwan , Adulto Jovem
6.
Acta Derm Venereol ; 100(1): adv00017, 2020 01 07.
Artigo em Inglês | MEDLINE | ID: mdl-31742644

RESUMO

Mycosis fungoides and Sézary syndrome belong to the group of primary cutaneous T-cell lymphomas. Because of the inflammatory appearance of the skin lesions, we hypothesized that antimicrobial peptides might be dysregulated in these conditions, similar to in inflammatory skin conditions. Samples from 20 patients with cutaneous T-cell lymphoma were analysed using immunohistochemistry and enzyme-linked immunoassay (ELISA) of skin washing fluids of hBD-2, hBD-3, RNase 7 and psoriasin. Immunohistochemistry results were compared with previous analyses of healthy and psoriatic skin. ELISA and immunohistochemistry revealed a higher expression of psoriasin in lesional cutaneous T-cell lymphoma compared with non-lesional and healthy samples. Immunohistochemistry showed an increase in hBD-2 in lesional cutaneous T-cell lymphoma skin compared with healthy skin. The expression profile of antimicrobial peptides in cutaneous T-cell lymphoma appears to be dysregulated, indicating a potential role of antimicrobial peptides in cutaneous T-cell lymphoma. A larger prospective study and functional studies are needed to improve our understanding of the role of antimicrobial peptides in cutaneous T-cell lymphoma.


Assuntos
Linfoma Cutâneo de Células T/genética , Neoplasias Cutâneas/genética , Pele/patologia , Adulto , Idoso , Feminino , Humanos , Imuno-Histoquímica , Linfoma Cutâneo de Células T/patologia , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Neoplasias Cutâneas/patologia
7.
J Dermatolog Treat ; 31(1): 91-98, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30892982

RESUMO

Introduction: The National Comprehensive Cancer Network and the European Organization for Research and Treatment of Cancer recommend extracorporeal photopheresis (ECP) as systemic therapy for cutaneous T-cell lymphoma (CTCL).Objective: To investigate real-world use of ECP in CTCL patients in the US.Methods: Data from the Truven MarketScan® database (2010-2015) were used to create a cohort of CTCL patients receiving systemic treatment. Multivariable regressions were performed to compare health care resource utilization between ECP and propensity score-matched non-ECP patients.Results: Of the 1106 eligible patients, 117 (10.6%) received ECP, with an average treatment duration of 13.6 months. Psoriasis, organ transplant, graft versus host disease, and scleroderma were the most common comorbidities. ECP was used as monotherapy in 76 patients (65.0%) and combination in 41 patients (35.0%), mostly with interferon and/or a retinoid. Higher Charlson Comorbidity Index (2.6 vs 2.2, p < .05), rates of organ transplant (49.6% vs 7.8%, p < .001), and graft vs host disease (41.9% vs 3.4%, p < .001) were observed in ECP versus non-ECP patients. Post-matching analyses showed that ECP patients had shorter all-cause inpatient stay (6.67 vs 11.80 days, p = .001).Conclusions: Approximately 1 out of 10 CTCL patients receiving systemic treatment were on ECP treatment in the US. Post-matching analysis showed ECP was associated with a shorter hospital stay.


Assuntos
Linfoma Cutâneo de Células T/terapia , Fotoferese , Adolescente , Adulto , Idoso , Comorbidade , Bases de Dados Factuais , Circulação Extracorpórea , Feminino , Humanos , Interferons/uso terapêutico , Linfoma Cutâneo de Células T/tratamento farmacológico , Linfoma Cutâneo de Células T/patologia , Masculino , Pessoa de Meia-Idade , Retinoides/uso terapêutico , Estados Unidos , Adulto Jovem
8.
Dermatol Ther ; 33(1): e13200, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31854482

RESUMO

We present a 32-year-old man with successful treatment and remission of mycosis fungoides of both axillae in 2016 after PUVA therapy and systemic and local administration of corticosteroids. Subsequently, in 2017, the patient also achieved remission of a T-cell CD 30 positive, ALK-1 negative large-cell lymphoma of a retroperitoneal and inguinal lymph node after chemotherapy and radiotherapy. One year later, in 2018, the patient presented to our clinic with progression of skin lesions in both axillary areas and the appearance of а tumor in the right gluteal region.Dermatological examination showed livid-to-erythematous, partly sclerotic plaques in the right inguinal area, cutis laxa-like plaque formations in the right axillary region with similar but less-developed changes in the left axillary fold, a solitary subcutaneous tumor formation affecting the entire right gluteal region, and enlarged, palpable lymph nodes in the right para-axillary area. Biopsies were obtained from an axillary lesion and the surgically removed axillary lymph nodes, and histological examination revealed changes of granulomatous slack skin in the axilla and reactive inflammatory changes in the lymph nodes. Histology of gluteal tissue showed a "foreign body" type of reaction with sarcoid-like features, where the patient in the past have been injected with anabolic and steroidal drugs. Herein we describe a patient with simultaneous occurrence of granulomatous slack skin type mycosis fungoides and a sarcoid-like reaction. The question remains open whether this represents the so-called sarcoidosis-lymphoma syndrome or, more likely, granulomatous slack skin MF associated with a sarcoid-like reaction of "foreign body" type. The possibility that disturbance of tissue homeostasis by incorporation of certain adjuvants within injections (for example) in the past might have been an inducer of cutaneous T cell lymphoma and sarcoidosis/sarcoid like lesions seems reasonable but also speculative.


Assuntos
Anabolizantes/efeitos adversos , Linfoma Cutâneo de Células T/etiologia , Micose Fungoide/etiologia , Sarcoidose/etiologia , Adulto , Anabolizantes/administração & dosagem , Humanos , Linfoma Cutâneo de Células T/diagnóstico , Linfoma Cutâneo de Células T/patologia , Masculino , Micose Fungoide/diagnóstico , Micose Fungoide/patologia , Sarcoidose/diagnóstico , Sarcoidose/patologia
9.
G Ital Dermatol Venereol ; 154(6): 681-695, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31859467

RESUMO

Cutaneous T cell lymphoma (CTCL) is a non-Hodgkin's lymphoma with a heterogenous presentation and highly variable disease course. The most common subtypes of CTCL are mycosis fungoides (MF) and Sézary Syndrome (SS). Treatment varies based on the stage of the disease with skin directed therapies typically utilized for early stage disease, and systemic therapies employed for more advanced disease. There are few highly effective treatments available, and systemic therapies have limited response rates. Histone deacetylase inhibitors have emerged as mainstream treatments for MF/SS over the past several years. Here, we discuss the mechanism of action of histone deacetylase inhibitors in relation to the pathogenesis of MF/SS, evaluate the clinical trials that led to Food and Drug Administration approval of two of the histone deacetylase inhibitors for MF/SS and describe the results for those still under investigation. Additionally, we discuss the potential for combination therapies in order to optimize outcomes of treatment with histone deacetylase inhibitors.


Assuntos
Inibidores de Histona Desacetilases/administração & dosagem , Linfoma Cutâneo de Células T/tratamento farmacológico , Neoplasias Cutâneas/tratamento farmacológico , Animais , Inibidores de Histona Desacetilases/farmacologia , Humanos , Linfoma Cutâneo de Células T/enzimologia , Linfoma Cutâneo de Células T/patologia , Micose Fungoide/tratamento farmacológico , Micose Fungoide/enzimologia , Micose Fungoide/patologia , Estadiamento de Neoplasias , Síndrome de Sézary/tratamento farmacológico , Síndrome de Sézary/enzimologia , Síndrome de Sézary/patologia , Neoplasias Cutâneas/enzimologia , Neoplasias Cutâneas/patologia
10.
Probl Radiac Med Radiobiol ; 24: 516-521, 2019 Dec.
Artigo em Inglês, Ucraniano | MEDLINE | ID: mdl-31841492

RESUMO

A clinical case of cutaneous T-cell lymphoma is presented in this paper - Mycosis fungoides, a tumorous stage that developed in a patient who suffered as a result of the Chornobyl accident. The most likely development of this di- sease is due to the influence of the radiation factor, which led to the affection of lymphoid cells. This disease has characteristic staging with a typical morphological pattern only in later stages, therefore, the diagnosis usually occurs in evident clinical and histological changes of the mycotic and tumorous stages, when treatment is ineffec- tive. The features of the disease and treatment are described.


Assuntos
Acidente Nuclear de Chernobyl , Socorristas , Linfoma Cutâneo de Células T/patologia , Micose Fungoide/patologia , Lesões por Radiação/patologia , Neoplasias Cutâneas/patologia , Diagnóstico Tardio , Dexametasona/uso terapêutico , Feminino , Humanos , Linfoma Cutâneo de Células T/diagnóstico , Linfoma Cutâneo de Células T/etiologia , Linfoma Cutâneo de Células T/terapia , Pessoa de Meia-Idade , Micose Fungoide/diagnóstico , Micose Fungoide/etiologia , Micose Fungoide/terapia , Estadiamento de Neoplasias , Doses de Radiação , Exposição à Radiação/efeitos adversos , Lesões por Radiação/diagnóstico , Lesões por Radiação/etiologia , Lesões por Radiação/terapia , Pele/patologia , Pele/efeitos da radiação , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/etiologia , Neoplasias Cutâneas/terapia , Sobreviventes , Terapia Ultravioleta
11.
Hematol Oncol ; 37(5): 569-577, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31674027

RESUMO

Romidepsin is a class I selective histone deacetylase (HDAC) inhibitor approved by the Food and Drug Administration (FDA) for relapsed/refractory (R/R) cutaneous T-cell lymphoma (CTCL) and peripheral T-cell lymphoma (PTCL), treated with at least one prior systemic therapy. Currently, there is paucity of real-life data on the efficacy and safety of romidepsin in R/R T-cell lymphoma. This national, multicenter study presents real-life data on the efficacy and safety of romidepsin in R/R T-cell lymphoma. Patients diagnosed and treated with romidepsin for R/R CTCL or PTCL between 2013 and 2018 were retrospectively reviewed. Outcomes included overall survival (OS), event-free survival (EFS), overall response rate (ORR), complete response (CR), and adverse events. Fifty-three patients with R/R PTCL (n = 42) or CTCL (n = 11) were included. Among CTCL patients, median OS was not reached, ORR was 25%, and none achieved CR. Among PTCL patients, median OS was 7.1 months, EFS was 1.9 months, ORR rate was 33%, and 12.5% achieved CR. In a univariate analysis, predictors for longer EFS include any response to therapy, number of previous lines, and PTCL subclass (with better results for angioimmunobalstic T-cell lymphoma). In a univariate and multivariate analysis for OS, treatment response was the only factor predicting OS (OR 4.48; CI 95%, 1.57-12.79; P = .005). Most grade 3 and 4 adverse events were hematological (35%). Infections were reported in 34% of patients. This real-life experience with romidepsin confirms the results of the pivotal phase II trials. PTCL subtype and the number of previous lines of therapy have an impact on EFS. In addition, patients who had good response to romidepsin benefited most in terms of both EFS and OS. Efforts should be done to identify those patients.


Assuntos
Antibióticos Antineoplásicos/uso terapêutico , Depsipeptídeos/uso terapêutico , Linfoma Cutâneo de Células T/tratamento farmacológico , Linfoma de Células T Periférico/tratamento farmacológico , Adulto , Idoso , Idoso de 80 Anos ou mais , Antibióticos Antineoplásicos/administração & dosagem , Antibióticos Antineoplásicos/efeitos adversos , Depsipeptídeos/administração & dosagem , Depsipeptídeos/efeitos adversos , Resistencia a Medicamentos Antineoplásicos , Feminino , Humanos , Estimativa de Kaplan-Meier , Linfoma Cutâneo de Células T/mortalidade , Linfoma Cutâneo de Células T/patologia , Linfoma de Células T Periférico/mortalidade , Linfoma de Células T Periférico/patologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Recidiva , Retratamento , Resultado do Tratamento
15.
Dermatol Online J ; 25(7)2019 Jul 15.
Artigo em Inglês | MEDLINE | ID: mdl-31450274

RESUMO

Granulomatous slack skin (GSS) is a rare subtype of mycosis fungoides. It usually presents as slowly evolving, erythematous, slack plaques that usually involve folds of lax skin. Herein, we report a case of GSS and we show electron microscopy examination. Atypical T cells with convoluted and cerebriform nuclei, lymphophagocytosis, and elastophagocytosis are key features of GSS under electron microscopy.


Assuntos
Derme/ultraestrutura , Linfoma Cutâneo de Células T/patologia , Microscopia Eletrônica , Neoplasias Cutâneas/patologia , Adulto , Biópsia , Diagnóstico Diferencial , Humanos , Linfoma Cutâneo de Células T/diagnóstico , Masculino , Micose Fungoide/diagnóstico , Pele/patologia , Neoplasias Cutâneas/diagnóstico
16.
Dermatol Online J ; 25(7)2019 Jul 15.
Artigo em Inglês | MEDLINE | ID: mdl-31450279

RESUMO

Angioimmunoblastic T-cell lymphoma (AITL) is a rare form of non-Hodgkin lymphoma often accompanied by autoimmune and paraneoplastic phenomena. Up to 50% of patients with AITL present with skin manifestations. This case series highlights two cases of AITL presenting with unusual cutaneous findings: one with a medium-vessel vasculitis and another with a chronic urticarial eruption. Clinicians should consider AITL in the differential diagnosis of vasculitis or urticaria refractory to standard treatment.


Assuntos
Linfoma Cutâneo de Células T/complicações , Síndromes Paraneoplásicas/patologia , Neoplasias Cutâneas/complicações , Pele/patologia , Urticária/etiologia , Vasculite/etiologia , Evolução Fatal , Feminino , Humanos , Linfadenopatia Imunoblástica/patologia , Linfoma Cutâneo de Células T/patologia , Pessoa de Meia-Idade , Neoplasias Cutâneas/patologia , Urticária/patologia , Vasculite/patologia
17.
J Cutan Pathol ; 46(12): 913-924, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31403211

RESUMO

BACKGROUND: Sézary syndrome (SS) and erythrodermic mycosis fungoides (E-MF) represent two expressions of erythrodermic cutaneous T-cell lymphoma (E-CTCL). METHODS: Histopathologic features were compared on skin specimens from 41 patients with SS and 70 patients with E-MF. Immunopathologic findings were compared on 42 SS and 79 E-MF specimens. RESULTS: Specimens of SS usually showed band-like dermal infiltrates with intermediate-sized lymphoid cells and few plasma cells; on the other hand E-MF more often had a perivascular infiltrative pattern, predominance of small/mixed lymphoid cells and eosinophils. SS also had lower numbers of CD8+ cells and higher numbers of CD62L+ cells compared to E-MF. For E-MF patients, the presence of large Pautrier collections, infiltrates with intermediate-sized cells, increased number of mitotic figures and ≥50% CD62L+ cells in the dermal infiltrate correlated with a relatively poor disease-specific survival. However, only the presence of mitotic figures retained prognostic significance with clinical stage as a covariate. CONCLUSIONS: Clinical stage provides the most important prognostic information for patients with E-CTCL. However, mitotic activity for E-MF and CD8+ cells <20% for SS have additional value. We hypothesize that observed differences in plasma cell and eosinophil numbers may reflect the influence of CD62L+ central memory T-cells in the microenvironment.


Assuntos
Linfoma Cutâneo de Células T/imunologia , Linfoma Cutâneo de Células T/patologia , Neoplasias Cutâneas/imunologia , Neoplasias Cutâneas/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Linfócitos T CD8-Positivos/imunologia , Eosinófilos/imunologia , Feminino , Humanos , Selectina L/imunologia , Masculino , Pessoa de Meia-Idade , Micose Fungoide/imunologia , Micose Fungoide/patologia , Prognóstico , Estudos Retrospectivos , Síndrome de Sézary/imunologia , Síndrome de Sézary/patologia , Microambiente Tumoral/imunologia
18.
Expert Opin Investig Drugs ; 28(9): 799-809, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31398295

RESUMO

Introduction: Cutaneous T-cell lymphoma (CTCL) is a heterogeneous group of skin-homing T-cell neoplasms, which represent approximately 75% of all primary cutaneous lymphomas. Mycosis fungoides and Sézary syndrome are the most common CTCL. Early stage disease follows a protracted course, carries a 5-year disease specific survival of 97% and can be treated with skin-directed therapies. Widespread, advanced disease has a 5-year OS of less than 25% and necessitates systemic treatment. Allogeneic stem cell transplantation is a potentially curative treatment option for advanced CTCL, however, transplant-related morbidity and mortality must be considered and a risk-benefit assessment performed on individual basis. Areas covered: Herein, we provide a review of investigative drugs in early-stage trials for the treatment of cutaneous CTCL, including topically applied immunomodulators such as replicating herpes virus or toll-like receptor 7/8 agonist resiquimod and systemic therapies with monoclonal antibodies, such as anti-CD47, recombinant cytotoxic interleukin 2 fusion protein anti-KIR3DL2 antibody and anti-miR-155 antibody. Expert Opinion: Among the reviewed drugs, resiquimod shows promising clinical efficacy with good tolerability in early CTCL. In refractory or relapsed disease, intratumoral anti-CD47-, anti-CCR4- and anti-KIR3DL2-antibodies show high response rates, however, latter two also show considerable toxicity. Larger trials are needed to better evaluate the discussed therapies.


Assuntos
Antineoplásicos Imunológicos/farmacologia , Linfoma Cutâneo de Células T/tratamento farmacológico , Neoplasias Cutâneas/tratamento farmacológico , Animais , Antineoplásicos Imunológicos/efeitos adversos , Desenvolvimento de Medicamentos/métodos , Drogas em Investigação/efeitos adversos , Drogas em Investigação/farmacologia , Transplante de Células-Tronco Hematopoéticas/métodos , Humanos , Linfoma Cutâneo de Células T/patologia , Micose Fungoide/tratamento farmacológico , Micose Fungoide/patologia , Estadiamento de Neoplasias , Síndrome de Sézary/tratamento farmacológico , Síndrome de Sézary/patologia , Neoplasias Cutâneas/patologia , Taxa de Sobrevida
19.
Dermatol Clin ; 37(4): 455-469, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31466586

RESUMO

Cutaneous T cell lymphoma (CTCL) represents a heterogeneous group of extranodal non-Hodgkin lymphomas in which monoclonal T lymphocytes infiltrate the skin. The mechanism of CTCL development is not fully understood, but likely involves dysregulation of various genes and signaling pathways. A variety of treatment modalities are available, and although they can induce remission in most patients, the disease may recur after treatment cessation. Owing to relatively low incidence and significant chronicity of disease, and the high morbidity of some therapeutic regimens, further clinical trials are warranted to better define the ideal treatment option for each subtype of CTCL.


Assuntos
Linfoma Cutâneo de Células T/diagnóstico , Neoplasias Cutâneas/diagnóstico , Antineoplásicos/uso terapêutico , Biópsia , Rearranjo Gênico do Linfócito T , Genes Codificadores dos Receptores de Linfócitos T , Humanos , Imunofenotipagem , Linfoma Cutâneo de Células T/genética , Linfoma Cutâneo de Células T/patologia , Linfoma Cutâneo de Células T/terapia , Fotoferese , Fototerapia , Neoplasias Cutâneas/genética , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/terapia
20.
Am J Dermatopathol ; 41(9): 644-648, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31433793

RESUMO

The recently published 2016 revision of the WHO classification of lymphoid neoplasms includes primary cutaneous acral CD8-positive T-cell lymphoma (PCATCL) as a provisional entity. This is a rare indolent lymphoma characterized by papules or nodules on the ear and a dermal infiltrate of CD8-positive T-lymphocytes with cytotoxic marker expression. A retrospective review of a single institutional experience with PCATCL identified 3 patients (mean age 54; range 49-62) with papules or nodules on the ear. Lesional biopsies demonstrated a dense diffuse dermal infiltrate of atypical lymphocytes with a Grenz zone in 2 cases and focal epidermotropism in 1 case. The atypical lymphocytes were predominantly CD3 and CD8 positive with expression of cytotoxic marker TIA1. Staging evaluation failed to reveal systemic disease. Two patients underwent local excision, and the third received local radiation therapy all with complete response and no disease recurrence at last follow-up 3 months (range 2-5 months). Our cases add to the existing limited literature on the clinical and histopathological features of PCATCL. We also performed an updated systematic literature view of the entity.


Assuntos
Linfócitos T CD8-Positivos/patologia , Linfoma Cutâneo de Células T/patologia , Linfoma Cutâneo de Células T/terapia , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/terapia , Biópsia por Agulha , Procedimentos Cirúrgicos Dermatológicos/métodos , Humanos , Imuno-Histoquímica , Linfoma Cutâneo de Células T/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons/métodos , Prognóstico , Radioterapia/métodos , Estudos Retrospectivos , Amostragem , Neoplasias Cutâneas/diagnóstico por imagem , Resultado do Tratamento
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