RESUMO
BACKGROUND: Mycosis fungoides is the most frequent form of cutaneous T-cell lymphoma. It is characterized by a chronic, slow, and progressive course, and is associated with mortality rates that depend on several factors, such as clinical staging. A median survival time of up to 13 months is found in patients with advanced stages that require more aggressive treatments, with greater toxicity and higher costs. In Latin America, few prognostic studies of the disease are available. OBJECTIVE: To determine the rate of progression from early stages (IA, IB, IIA) to more advanced stages (> IIB) in patients older than 18 years with mycosis fungoides treated at two medical centers in Colombia between January 1, 2010, and December 31, 2019. METHODS: Retrospective cohort study with a longitudinal design. RESULTS: 112 patients diagnosed with early mycosis fungoides were included. 56.2% were male (n = 63), with a median age of 53 years (IQR 43â67). The most frequent clinical variant was classic (67.9%; n = 76), followed by folliculotropic (16%; n = 18), and hypopigmented (10.7%; n = 12). The most common first-line treatment was NB-UVB phototherapy (27.7%; n = 31), followed by PUVA phototherapy (25.8%; n = 29%), and topical corticosteroids (25%; n = 28). The global rate of disease progression was 8% (n = 9), with an overall mortality of 12.5% (n = 14). STUDY LIMITATIONS: Its retrospective design and the lack of molecular studies for case characterization. CONCLUSIONS: Early mycosis fungoides is a disease with a good prognosis in most patients, with a progression rate of 8% (n = 9).
Assuntos
Progressão da Doença , Micose Fungoide , Estadiamento de Neoplasias , Neoplasias Cutâneas , Humanos , Micose Fungoide/patologia , Micose Fungoide/terapia , Micose Fungoide/mortalidade , Masculino , Feminino , Estudos Retrospectivos , Pessoa de Meia-Idade , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/mortalidade , Neoplasias Cutâneas/terapia , Adulto , Idoso , Colômbia/epidemiologia , Estudos Longitudinais , Fatores de Risco , Prognóstico , Terapia PUVA , Fatores de Tempo , Terapia UltravioletaRESUMO
Abstract Background Mycosis fungoides is the most frequent form of cutaneous T-cell lymphoma. It is characterized by a chronic, slow, and progressive course, and is associated with mortality rates that depend on several factors, such as clinical staging. A median survival time of up to 13 months is found in patients with advanced stages that require more aggressive treatments, with greater toxicity and higher costs. In Latin America, few prognostic studies of the disease are available. Objective To determine the rate of progression from early stages (IA, IB, IIA) to more advanced stages (> IIB) in patients older than 18 years with mycosis fungoides treated at two medical centers in Colombia between January 1, 2010, and December 31, 2019. Methods Retrospective cohort study with a longitudinal design. Results 112 patients diagnosed with early mycosis fungoides were included. 56.2% were male (n = 63), with a median age of 53 years (IQR 43‒67). The most frequent clinical variant was classic (67.9%; n = 76), followed by folliculotropic (16%; n = 18), and hypopigmented (10.7%; n = 12). The most common first-line treatment was NB-UVB phototherapy (27.7%; n = 31), followed by PUVA phototherapy (25.8%; n = 29%), and topical corticosteroids (25%; n = 28). The global rate of disease progression was 8% (n = 9), with an overall mortality of 12.5% (n = 14). Study limitations Its retrospective design and the lack of molecular studies for case characterization. Conclusions Early mycosis fungoides is a disease with a good prognosis in most patients, with a progression rate of 8% (n = 9).
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La leucemia/linfoma T del adulto es una neoplasia maligna de mal pronóstico frecuente en población anciana. Se presenta el caso de una mujer de 44 años de edad, de Ayacucho, diagnosticada con el subtipo linfomatoso de esta enfermedad e infección por virus linfotrópico T humano-I; mostró síndrome oclusivo de vena cava superior con tratamiento de quimioterapia sistémica bajo régimen de dosis ajustada con rituximab más etoposido, prednisona, vincristina, ciclofosfamida y doxorubicina. Posteriormente ingresó en emergencia por presentar dificultad respiratoria, tos seca, disminución de la conciencia, hipercalcemia, tomografía de tórax con patrón heterogéneo consolidativo en ambos pulmones y PCR en hisopado nasofaríngeo positivo a COVID-19. Recibió tratamiento de hidroxicloroquina, azitromicina, corticoides e ivermectina con pobre respuesta, rápido deterioro y fallece días después. La leucemia/linfoma T del adulto a edad temprana es rara y está relacionada con infecciones crónicas como strongyloides o tuberculosis, susceptible ante el padecimiento de COVID-19.
Adult T cell leukemia-lymphoma is a common malignancy with a poor prognosis in the elderly population. We present a 44-year-old woman from Ayacucho who was diagnosed with a lymphoma subtype of this disease and a human T-lymphotropic virus-I infection; she showed superior vena cava occlusive syndrome with systemic chemotherapy treatment under an adjusted-dose regimen with rituximab plus etoposide, prednisone, vincristine, cyclophosphamide and doxorubicin. Subsequently, she was admitted to the emergency service due to respiratory distress, dry cough, decreased consciousness, hipercalcemia, chest tomography with a heterogeneous consolidation pattern in both lungs and positive RT-PCR nasopharyngeal swab test for COVID-19. She received treatment with hydroxychloroquine, azithromycin, corticosteroids and ivermectin with a poor response, rapid deterioration and died later. Adult T cell leukemia-lymphoma at an early age is rare and is related to chronic infections such as strongyloides or tuberculosis, susceptible to COVID-19.
Assuntos
Linfoma de Células T , Infecções por Coronavirus , Herpesvirus Humano 6 , NeoplasiasRESUMO
A Leucemia/Linfoma de Células-T do Adulto (LLCT) é um tipo agressivo de doença linfoproliferativa causada pelo Vírus Linfotrópico de Células-T Humano (HTLV-1), classificada em cinco tipos: indolente, tumoral primária de pele, crônico, linfomatoso e agudo. O HTLV pertence à família Retroviridae, gênero Deltaretrovirus, subfamília Oncovirinae, de três genes estruturais (Gag, Pol e Env) e sua transmissão ocorre por contato sexual, transfusão de sangue ou inoculação por materiais perfuro- cortantes contaminados e aleitamento materno. Esta revisão narrativa tem como objetivo apresentar uma síntese sobre a relação do HTLV-1 na LLCT, seu processo patogênico e uma abordagem social. O vírus infecta, principalmente, células T CD4+, desregulando o sistema imunológico do hospedeiro, podendo ocasionar neoplasia de células-T a partir de uma única célula que teve expansão clonal, constituindo uma população monoclonal em que todas as células contêm o DNA proviral do HTLV-1. O prognóstico é ruim, a maioria dos pacientes que desenvolvem LLCT apresenta doença de progressão rápida e o óbito em curto período, mesmo com quimioterapia agressiva. Não há política nacional específica para o HTLV, não faz parte da lista de doenças de notificação compulsória e pouco se debate publicamente, gerando desconhecimento por parte da população e por parte de profissionais de saúde, sendo uma infecção negligenciada. Conclui-se que a infecção pelo HTLV é um tema que precisa ser popularizado, havendo urgência na implantação de políticas públicas específicas e de pesquisas contínuas, devido à grande incidência no Brasil e associação a doenças graves como a LLCT.
Adult T-Cell Leukemia/Lymphoma (TCLL) is an aggressive type of lymphoproliferative disease caused by the Human T-Cell Lymphotropic Virus (HTLV- 1), classified into five types: indolent, primary skin tumor, chronic, lymphomatous and sharp. HTLV belongs to the Retroviridae family, Deltaretrovirus genus, Oncovirinae subfamily, with three structural genes (Gag, Pol and Env) and its transmission occurs through sexual contact, blood transfusion or inoculation with contaminated sharps and material and breastfeeding. This narrative review aims to present a synthesis about the relationship of HTLV-1 in CLL, its pathogenic process and a social approach. The virus mainly infects CD4+ T cells, disrupting the host's immune system, and may cause T-cell neoplasia from a single cell that underwent clonal expansion, constituting a monoclonal population in which all cells contain the HTLV proviral DNA -1. Prognosis is poor, most patients who develop TCLL have rapidly progressive disease and death within a short period, even with aggressive chemotherapy. There is no specific national policy for HTLV, it is not part of the list of notifiable diseases and little is discussed publicly, generating ignorance on the part of the population and on the part of health professionals, being a neglected infection. It is concluded that HTLV infection is a topic that needs to be popularized, with urgency in the implementation of specific public policies and continuous research, due to the high incidence in Brazil and association with serious diseases such as TCLL.
La leucemia/linfoma de células T del adulto (LLCT) es un tipo agresivo de enfermedad linfoproliferativa causada por el virus linfotrópico de células T humanas (HTLV-1), clasificada en cinco tipos: indolente, tumor primario de piel, crónica, linfomatosa y aguda. El HTLV pertenece a la familia Retroviridae, género Deltaretrovirus, subfamilia Oncovirinae, con tres genes estructurales (Gag, Pol y Env) y su transmisión se da por contacto sexual, transfusión de sangre o inoculación con material cortopunzante contaminado y lactancia materna. Esta revisión narrativa tiene como objetivo presentar una síntesis sobre la relación del HTLV-1 en la LLC, su proceso patogénico y un abordaje social. El virus infecta principalmente a los linfocitos T CD4+, alterando el sistema inmunitario del huésped y puede causar neoplasia de linfocitos T a partir de una sola célula que experimentó expansión clonal, constituyendo una población monoclonal en la que todas las células contienen el ADN proviral -1 del HTLV. El pronóstico es malo, la mayoría de los pacientes que desarrollan LLCT tienen una enfermedad rápidamente progresiva y mueren en un período corto, incluso con quimioterapia agresiva. No existe una política nacional específica para el HTLV, no forma parte de la lista de enfermedades de notificación obligatoria y poco se discute públicamente, generando desconocimiento por parte de la población y por parte de los profesionales de la salud, siendo una infección desatendida. Se concluye que la infección por HTLV es un tema que necesita ser popularizado, con urgencia en la implementación de políticas públicas específicas y de investigación continua, debido a la alta incidencia en Brasil y la asociación con enfermedades graves como la LLCT.
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El linfoma angioinmunoblástico de células T (LAIT) es un linfoma no Hodgkin poco frecuente, puede imitar a enfermedades autoinmunes y es de pobre pronóstico. Se presenta el caso de una mujer de 36 años con 3 años de enfermedad caracterizada por fiebre, artralgias y baja de peso. La paciente fue diagnosticada inicialmente como lupus eritematoso sistémico, pero al no encontrar mejoría con el tratamiento su diagnóstico fue replanteado. En una nueva hospitalización se le identificaron múltiples adenomegalias. Se realizó la biopsia de una de las adenomegalias, la patología fue compatible con LAIT. Se indicó 3 sesiones de quimioterapia, sin embargo, desarrolló falla multiorgánica con desenlace fatal. El LAIT es un reto diagnóstico debido a que puede imitar varias patologías autoinmunes. Es muy importante su sospecha y descarte para iniciar un tratamiento precoz que mejore la sobrevida de los pacientes.
Angioimmunoblastic T-cell lymphoma (LAIT) is a rare non-Hodgkin lymphoma, can mimic autoimmune diseases, and has a poor prognosis. We present the case of a 36-year-old woman with a 3-year illness characterized by fever, arthralgia and weight loss. She was initially diagnosed as systemic lupus erythematosus, but finding no improvement with treatment, her diagnosis was reconsidered. In a new hospitalization, multiple lymph nodes were identified. They performed a biopsy of one of the adenopathies, the pathology was compatible with LAIT. Three chemotherapy sessions were indicated, however, she developed multiple organ failure with a fatal outcome. LAIT is a diagnostic challenge because it can mimic several autoimmune pathologies. Its suspicion and ruling out is very important to initiate early treatment that improves patient survival.
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Peripheral neuropathy (PN) is characterized by the injury to the peripheral nervous system of varied etiology. Lymphoma is one of the etiologies of PN, presenting various neurological manifestations. Neuropathy associated with peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS) is unusual and fewer cases are documented in the literature. In addition, PTCL, NOS is extremely rare as primary in the female genital tract, especially uterine cervix, and exhibits aggressive clinical course with poor therapy response. We hereby describe a 47-year-old female who presented with fever and chills for 15 days. Clinical examination revealed left-sided lower motor neuron type of facial nerve palsy with Bell's phenomenon. Nerve conduction study of all four limbs illustrated asymmetrical axonal neuropathy (motor > sensory), suggesting mononeuritis multiplex. She developed vaginal bleeding during her hospital stay. Pelvic examination and imaging revealed a 4x3cm polypoidal mass on the posterior lip of the cervix, which was excised and diagnosed as extranodal primary PTCL, NOS based on morphology, immunohistochemistry, and in-situ hybridization findings. Besides, the cerebrospinal fluid (CSF) was infiltrated by the lymphoma cells, detected on cell block preparation. The patient succumbed to her illness within one week despite best efforts and the commencement of chemotherapy. No consent was obtainable for nerve biopsy and autopsy. Thus, we report an extremely rare case of primary extranodal PTCL, NOS of the uterine cervix with unusual presentation of mononeuritis multiplex. Further, we discussed the differentials of PTCL, NOS at this extranodal site.
RESUMO
Peripheral neuropathy (PN) is characterized by the injury to the peripheral nervous system of varied etiology. Lymphoma is one of the etiologies of PN, presenting various neurological manifestations. Neuropathy associated with peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS) is unusual and fewer cases are documented in the literature. In addition, PTCL, NOS is extremely rare as primary in the female genital tract, especially uterine cervix, and exhibits aggressive clinical course with poor therapy response. We hereby describe a 47-year-old female who presented with fever and chills for 15 days. Clinical examination revealed left-sided lower motor neuron type of facial nerve palsy with Bell's phenomenon. Nerve conduction study of all four limbs illustrated asymmetrical axonal neuropathy (motor > sensory), suggesting mononeuritis multiplex. She developed vaginal bleeding during her hospital stay. Pelvic examination and imaging revealed a 4x3cm polypoidal mass on the posterior lip of the cervix, which was excised and diagnosed as extranodal primary PTCL, NOS based on morphology, immunohistochemistry, and in-situ hybridization findings. Besides, the cerebrospinal fluid (CSF) was infiltrated by the lymphoma cells, detected on cell block preparation. The patient succumbed to her illness within one week despite best efforts and the commencement of chemotherapy. No consent was obtainable for nerve biopsy and autopsy. Thus, we report an extremely rare case of primary extranodal PTCL, NOS of the uterine cervix with unusual presentation of mononeuritis multiplex. Further, we discussed the differentials of PTCL, NOS at this extranodal site.
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Neoplasias do Colo do Útero/complicações , Linfoma de Células T Periférico/complicações , Mononeuropatias/etiologia , Biópsia , Imuno-Histoquímica , Neoplasias do Colo do Útero/diagnóstico , Linfoma de Células T/diagnóstico , Hibridização In Situ , Evolução FatalRESUMO
Abstract Cutaneous T-cell lymphomas are a heterogeneous group of lymphoproliferative disorders, characterized by infiltration of the skin by mature malignant T cells. Mycosis fungoides is the most common form of cutaneous T-cell lymphoma, accounting for more than 60% of cases. Mycosis fungoides in the early-stage is generally an indolent disease, progressing slowly from some patches or plaques to more widespread skin involvement. However, 20% to 25% of patients progress to advanced stages, with the development of skin tumors, extracutaneous spread and poor prognosis. Treatment modalities can be divided into two groups: skin-directed therapies and systemic therapies. Therapies targeting the skin include topical agents, phototherapy and radiotherapy. Systemic therapies include biological response modifiers, immunotherapies and chemotherapeutic agents. For early-stage mycosis fungoides, skin-directed therapies are preferred, to control the disease, improve symptoms and quality of life. When refractory or in advanced-stage disease, systemic treatment is necessary. In this article, the authors present a compilation of current treatment options for mycosis fungoides and Sézary syndrome.
Assuntos
Humanos , Neoplasias Cutâneas/terapia , Linfoma Cutâneo de Células T , Micose Fungoide/terapia , Síndrome de Sézary/terapia , Qualidade de VidaRESUMO
Cutaneous T-cell lymphomas are a heterogeneous group of lymphoproliferative disorders, characterized by infiltration of the skin by mature malignant T cells. Mycosis fungoides is the most common form of cutaneous T-cell lymphoma, accounting for more than 60% of cases. Mycosis fungoides in the early-stage is generally an indolent disease, progressing slowly from some patches or plaques to more widespread skin involvement. However, 20% to 25% of patients progress to advanced stages, with the development of skin tumors, extracutaneous spread and poor prognosis. Treatment modalities can be divided into two groups: skin-directed therapies and systemic therapies. Therapies targeting the skin include topical agents, phototherapy and radiotherapy. Systemic therapies include biological response modifiers, immunotherapies and chemotherapeutic agents. For early-stage mycosis fungoides, skin-directed therapies are preferred, to control the disease, improve symptoms and quality of life. When refractory or in advanced-stage disease, systemic treatment is necessary. In this article, the authors present a compilation of current treatment options for mycosis fungoides and Sézary syndrome.
Assuntos
Linfoma Cutâneo de Células T , Micose Fungoide , Síndrome de Sézary , Neoplasias Cutâneas , Humanos , Micose Fungoide/terapia , Qualidade de Vida , Síndrome de Sézary/terapia , Neoplasias Cutâneas/terapiaRESUMO
Peripheral T-Cell Lymphoma Not Otherwise Specified it is a rare type of Non-Hodgkin t-cells malignant tumor whose oral manifestations are difficult to diagnose. A case of a 48-year-old male with a hemi-maxillary lesion histological and immunohistochemically compatible with Peripheral T-Cell Lymphoma not otherwise specified is presented. A case of a 48-year-old male with a hemi-maxillary lesion histological and immunohistochemically compatible with Peripheral T-Cell Lymphoma not otherwise specified is presented. The patient treatment consisted of chemotherapy, but after the second cycle, died from immunosuppressive complications. Early stage diagnosis of oral lesions is imperative to avoid aggressive treatment and low overall survival rate of such pathologies.
Introducción: El linfoma periférico de células T no especificado es un tipo raro de tumor maligno no Hodgkin de células T cuyas manifestaciones orales son difíciles de diagnosticar. Se presenta el caso de un varón de 48 años con lesión hemimaxilar histológica e inmunohistoquímicamente compatible con linfoma periférico de células T no especificado. El tratamiento del paciente consistió en quimioterapia, pero después del segundo ciclo, falleció por complicaciones inmunosupresoras. El diagnóstico temprano de las lesiones orales es imperativo para evitar un tratamiento agresivo y la baja tasa de supervivencia.
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Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Bucais/diagnóstico , Neoplasias Bucais/terapia , Linfoma de Células T Periférico/diagnóstico , Linfoma de Células T Periférico/terapia , Protocolos de Quimioterapia Combinada Antineoplásica , Diagnóstico Precoce , Tratamento FarmacológicoRESUMO
ABSTRACT Ocular adnexal involvement in CD30+ lymphoproliferative disorders is rare. We report the case of a 73-year-old woman with a relapsing primary cutaneous anaplastic large cell lymphoma on her eyelid. A systemic extension study excluded extracutaneous involvement. Systemic chemotherapy resulted in an optimal response, with complete regression of the cutaneous lesions. There has been no recurrence during the 2 years of follow-up.
RESUMO O acometimento ocular adicional nos distúrbios linfoproliferativos CD30+ é raro. Relatamos o caso de uma mulher de 73 anos com linfoma de grandes células anaplásicas primárias recidivantes em sua pálpebra. A avaliação sistêmica excluiu envolvimento extracutâneo. A quimioterapia sistémica resultou em uma resposta ótima, com regressão completa das lesões cutáneas. Não houve recidiva durante 2 anos de acompanhamento.
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Humanos , Feminino , Idoso , Linfoma Anaplásico de Células Grandes/patologia , Neoplasias Palpebrais/patologia , Biópsia , Resultado do Tratamento , Linfoma Anaplásico de Células Grandes/tratamento farmacológico , Neoplasias Palpebrais/tratamento farmacológicoRESUMO
Abstract: Recently, the World Health Organization published the revised 4th edition of its classification of tumors of hematopoietic and lymphoid tissues. The present paper is a concise comparative review of the main primary cutaneous T-cell hematopoietic tumors, with emphasis on their immunohistochemical profiles.
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Humanos , Organização Mundial da Saúde , Linfoma Cutâneo de Células T/classificação , Imuno-Histoquímica , Linfoma Cutâneo de Células T/diagnóstico , Diagnóstico DiferencialRESUMO
Abstract: Background: Mycosis fungoides (MF) is the most common subtype of cutaneous T-cell lymphoma. TNMB system is the staging method used in MF, and it not only guides therapeutic management, but represents the main prognostic factor. In order to improve the prognostic evaluation, the Cutaneous Lymphoma International Prognostic Index (CLIPi) was proposed. Objective: To evaluate the performance of CLIPi score for prognostic analysis in patients with early stage MF. Methods: This is a retrospective cross-sectional observational study, with exploratory analysis. The outcome variables were disease progression and related death. Results: One hundred and two patients were stratified according to CLIPi score, being the majority classified as low risk. Patients with intermediate or high risk presented disease progression more frequently than those with low risk (PR: 1.2 / p = 0.004 / 95%CI: 1.0 - 1.6). The same did not occur with the variable related death. In addition, survival rates were not consistent with risk stratification. Study Limitations: Small sample and its retrospective analysis. Conclusions: Since CLIPi score was proposed, four other studies that we could consult showed conflicting results, similar to the present study. Further studies are necessary for a recommendation of its use.
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Cutâneas/patologia , Micose Fungoide/patologia , Prognóstico , Neoplasias Cutâneas/mortalidade , Brasil/epidemiologia , Estudos Transversais , Taxa de Sobrevida , Estudos Retrospectivos , Seguimentos , Linfoma Cutâneo de Células T/mortalidade , Linfoma Cutâneo de Células T/patologia , Micose Fungoide/mortalidade , Síndrome de Sézary/patologia , Progressão da Doença , Estadiamento de NeoplasiasRESUMO
Abstract: Background: Mycosis fungoides is the most common form of primary cutaneous lymphoma, with an indolent, slowly progressive course and 88% five-year survival rate. The diagnosis is challenging, especially in the early stages, and usually relies on a good clinical-histopathological correlation. Objective: The aim was to establish the clinical and epidemiological profile of patients with early-stage mycosis fungoides. Methods: This was a retrospective cross-sectional observational study with an exploratory analysis. Outcome variables were disease progression and mycosis fungoides-related death. Results: One hundred and two patients were included. The majority were white males, with a mean age of 55.6 years. Mean time from onset of lesions to diagnosis was 51.08 months. The majority of patients were classified as IB stage according to TNMB. Mean follow-up time was 7.85 years. Disease progression was seen in 29.4% of the patients. Death related to the disease occurred in 7.9% of patients. Plaque lesions, involvement of more than 10% of the body surface, altered lactate dehydrogenase and beta-2-microglobulin, and stage IB were significantly associated with disease progression, and altered lactate dehydrogenase and beta-2-microglobulin also correlated with higher frequency of deaths. Study limitations: Small sample and retrospective design. Conclusions: The clinical and epidemiological profile of patients with early-stage mycosis fungoides in our sample corroborates reports in the literature. Diagnostic delay in our series is also consistent with previous findings, but the rate of disease progression, despite treatment, was higher than reported in the literature.
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Humanos , Masculino , Feminino , Adolescente , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Adulto Jovem , Neoplasias Cutâneas/patologia , Micose Fungoide/patologia , Neoplasias Cutâneas/epidemiologia , Brasil/epidemiologia , Criança , Prevalência , Estudos Transversais , Estudos Retrospectivos , Micose Fungoide/epidemiologia , Progressão da Doença , Estadiamento de NeoplasiasRESUMO
Introducción: Los linfomas son neoplasias del sistema linfoide clasificadas en Hodgkin y No-Hodgkin. Los linfomas de cabeza y cuello se originan en tejido linfoide regional, pero también en otros sitios como encía, paladar, etc. Objetivo: Describir el Linfoma Anaplásico de Células Grandes (LACG) como un subgrupo de linfomas No-Hodkin de células T, positivo o negativo para la expresión de las proteínas CD30 y AKL, el cual puede afectar numerosas partes del cuerpo incluyendo la cavidad oral. Se reporta un caso de LACG de células T CD30+ALK+. Diseño: Reporte de caso. Metodología: Se realizó una búsqueda sistemática en PubMed, MEDLINE, EMBASE, LILACS y Ovid. Se seleccionaron los artículos que reportaron casos de Linfoma No-Hodkin de células T con manifestaciones orales, limitándose al idioma Inglés. Reportamos el caso de una paciente de 9 años con LACG de células T, CD30+ALK+ con manifestaciones orales y sistémicas. Resultados: De los artículos obtenidos ninguno reporta casos de LACG de células T CD30+ ALK+ con manifestaciones orales, por la cual no fue posible realizar el análisis sistemático. Discusión: El LACG representa 2-3% de los Linfomas No-Hodkin, se divide en cutáneo primario y sistémico (ALK+/ALK-). El ALK+ también puede afectar sitios extranodales. Los pacientes a menudo presentan síntomas sistémicos, como fiebre y sudoración nocturna. Los casos de LACG sistémico primario ALK+ suelen responder bien a la quimioterapia y tienen mejor pronóstico que los casos ALK-. Conclusiones: El Linfoma anaplásico de células grandes de células T, CD30+ALK+ es una entidad poco frecuente en la cavidad oral.
Introduction: Lymphomas are neoplasms of the lymphoid system, they are classified as Hodgkin and Non-Hodgkin. Head and neck lymphomas are originated in regional lymphoid tissue, but also in other sites such as gums, lips, palate, etc. Objective: To describe the Anaplastic Large Cell Lymphoma (ALCL) as a subgroup of T-cell non-Hodgkin's lymphoma, positive or negative for expression of CD30+ and AKL+proteins, which can affect many parts of the body including tissue of oral cavity. Finally, it is pretended to report a case of ALCL T-cell, CD30+ AKL+. Design: Case report. Materials and methods: A systematic search was performed using electronic databases such as PUBMED, MEDLINE, EMBASE, LILACS and Ovid. Those papers which reported cases of T-cell NHL with oral manifestations were selected for further evaluation. This search was limited to english language. Additionally, the case of an ALCL T-cell, CD30+AKL+ with oral and systemic manifestations in a 9-year-old girl treated at the Fundación Hospital de la Misericordia is reported. Results: Of the articles obtained none report cases of ALCL T-cell, CD30+AKL+ with oral manifestations, so it is not possible to perform a systematic review of them. Discussion: ALCL is classified as primary cutaneous and systemic (ALK + and ALK-). The ALCL represents 2-3% of all NHL. The ALK+ ALCL can also affect extranodal sites. Patients often have systemic symptoms, such as fever and night sweats. Cases of ALK+ primary systemic ALCL usually respond well to chemotherapy and have a better prognosis compared to ALK- cases which have a less favorable prognosis with unpredictable clinical behavior. Conclusions: ALCL T-cell, CD30+AKL+ is an entity with presentation rare in the oral cavity.
Assuntos
Humanos , Transtornos Linfoproliferativos , Linfoma de Células T , Linfoma Anaplásico de Células GrandesRESUMO
Abstract: Background: Primary cutaneous T-cell lymphomas constitute a heterogeneous and rare group of diseases with regional particularities in Latin America. Objective: To determine the clinicopathological features, relative frequency and survival among patients from a Peruvian institution. Methods: Primary cutaneous T-cell lymphomas were defined based on the absence of extracutaneous disease at diagnosis. Classification was performed following the 2008 World Health Organization Classification of Neoplasms of the Hematopoietic and Lymphoid tissues. Risk groups were established according to the 2005 World Health Organization-EORTC classification for cutaneous lymphomas. Data of patients admitted between January 2008 and December 2012 were analyzed. Results: 74 patients were included. Mean age was 49.5 years. In order of frequency, diagnoses were: mycosis fungoides (40.5%), peripheral T-cell lymphoma not otherwise specified (22.95%), adult T-cell lymphoma/leukemia (18.9%), CD30+ lymphoproliferative disorders (6.8%), hydroa vacciniforme-like lymphoma (5.4%), extranodal NK/T-cell lymphoma (4.1%) and Sézary syndrome (1.4%). Predominant clinical patterns were observed across different entities. Mycosis fungoides appeared mainly as plaques (93%). Peripheral T-cell lymphoma not otherwise specified and adult T-cell lymphoma/leukemia presentation was polymorphic. All patients with hydroa vacciniforme-like lymphoma presented with facial edema. All cases of extranodal NK/T-cell lymphoma appeared as ulcerated nodules/tumors. Disseminated cutaneous involvement was found in 71.6% cases. Forty-six percent of patients were alive at 5 years. Five-year overall survival was 76.4% and 19.2%, for indolent and high-risk lymphomas, respectively (p<0.05). High risk group (HR: 4.6 [2.08-10.18]) and increased DHL level (HR: 3.2 [1.57-6.46]) emerged as prognostic factors for survival. Study limitations: Small series. Conclusion: Primary cutaneous T-cell lymphomas other than mycosis fungoides or CD30+ lymphoproliferative disorders are aggressive entities with a poor prognosis.
Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Adulto Jovem , Neoplasias Cutâneas/epidemiologia , Peru/epidemiologia , Prognóstico , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/tratamento farmacológico , Análise de Sobrevida , Fatores de Risco , Linfoma Cutâneo de Células T/diagnóstico , Linfoma Cutâneo de Células T/tratamento farmacológico , Linfoma Cutâneo de Células T/epidemiologiaRESUMO
Lymphomatoid papulosis (LyP) is defined as a chronic, recurrent, self-healing papulonecrotic or papulonodular skin disease with histologic features suggestive of a (CD30-positive) malignant lymphoma. In up to 20% of patients, LyP are preceded by, associated with, or followed by another type of cutaneous or systemic lymphoma, generally mycosis fungoides (MF), primary cutaneous anaplastic large cell lymphoma (C-ALCL). In this case, we describe a case of MF that preceded and continued to coexist with LyP type C.(AU)
A papulose linfomatóide (LyP) é definida como uma doença cutânea papulonecrótica ou papulonodular crônica, recorrente, com características histológicas sugestivas de linfoma maligno (CD30-positivo). Em até 20% dos pacientes, o LyP é precedido por, associado ou seguido por outro tipo de linfoma cutâneo ou sistêmico, geralmente micose fungóide (MF), linfoma cutâneo primário de células grandes anaplásicas (C-ALCL). Neste caso, descrevemos um caso de MF que precedeu e continuou a coexistir com LyP tipo C. (AU)
Assuntos
Humanos , Feminino , Adulto , Linfoma , Linfoma Anaplásico Cutâneo Primário de Células Grandes , Papulose Linfomatoide , Micose Fungoide , Linfócitos TRESUMO
Abstract Mycosis fungoides is a cutaneous T-cell lymphoma with various clinical and pathological presentations. Early lesions are nonspecific, which hinders early diagnosis. The folliculotropic subtype is manifested as acneiform lesions, follicular papules or erythematous plaques mainly on the face, neck and upper trunk. Histopathology shows dense lymphocytic infiltrate surrounding and infiltrating the hair follicles. A case of difficult histopathological diagnosis with florid and unusual skin lesions mainly on the face is reported.
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Pele/patologia , Micose Fungoide/patologia , Neoplasias Cutâneas/patologia , Biópsia , Imuno-HistoquímicaRESUMO
Abstract: Sézary syndrome is a primary cutaneous T-cell lymphoma characterized by the triad of erythroderma, lymphadenopathy and circulating atypical cells. The emergence of new molecular targets has enabled the development of drugs such as alemtuzumab, an anti-CD52 monoclonal antibody, which has shown promising results in the treatment of this entity. We report the case of a 70-year-old male with refractory Sézary syndrome in whom treatment with alemtuzumab achieved an 80% skin lesion clearance with complete haematologic and radiologic response. The treatment was discontinued after 4 months due to adverse effects, with the patient showing a sustained response without disease progression after 13 months of follow-up.
Assuntos
Humanos , Masculino , Idoso , Neoplasias Cutâneas/tratamento farmacológico , Síndrome de Sézary/tratamento farmacológico , Anticorpos Monoclonais Humanizados/uso terapêutico , Antineoplásicos/uso terapêutico , Neoplasias Cutâneas/sangue , Contagem de Células Sanguíneas , Antígenos de Diferenciação de Linfócitos T/metabolismo , Síndrome de Sézary/sangue , Resultado do Tratamento , AlemtuzumabRESUMO
Abstract: Immunosuppressive drugs and biological agents may represent a potential risk of lymphoma development in patients with rheumatoid arthritis. But most cases are diffuse, large B-cell lymphomas. Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma, a provisional entity in the 2005 WHO-EORTC classification of cutaneous lymphomas, is only described in a limited number of reports. To our knowledge, our case is a rare instance of primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma, after associated treatment with methotrexate and etanercept, in a patient with moderate rheumatoid arthritis who had undergone an orchidectomy incorrectly.