RESUMO
BACKGROUND: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare primary cutaneous lymphoma of mature cytotoxic T cells. Initially, patients with SPTCL were treated with doxorubicin-based polychemotherapy. OBJECTIVE: To analyze clinical, biologic, immunophenotypical, molecular, imaging, treatment, and outcome data reflecting the current state of knowledge. METHODS: A retrospective multicenter study of 16 patients with SPTCL that was diagnosed between 1996 and 2016. RESULTS: The female-to-male ratio was 1.7. The median age at diagnosis was 46.5 years. Patients presented with multiple nodular or plaque-like lesions preferentially affecting the legs and/or trunk. Histopathology typically showed a lobular panniculitis with individual adipocytes surrounded by atypical lymphocytes, usually with a CD3+, CD4-, CD8+, CD56-, TIA1 cytotoxic granule associated RNA binding protein 1-positive phenotype and high proliferation rate. SPTCL was associated with autoimmune diseases in 25% of patients, and with the development of hemophagocytic syndrome in 18% of patients. Oral steroids alone or in combination with low-dose methotrexate or cyclosporine A were the most common initial treatment, achieving a complete response in 85% of the treated patients. The median follow-up time was 14 months. The 5-year disease-specific survival rate was 85.7%. LIMITATIONS: This was a retrospective study. CONCLUSIONS: SPTCL has an excellent prognosis. Immunosuppressive agents can be considered for first-line treatment.
Assuntos
Linfoma Cutâneo de Células T/patologia , Linfoma Cutâneo de Células T/terapia , Linfoma de Células T/patologia , Linfoma de Células T/terapia , Paniculite/patologia , Paniculite/terapia , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/terapia , Adulto , Idoso , Quimiorradioterapia/métodos , Estudos de Coortes , Intervalo Livre de Doença , Feminino , Humanos , Linfoma de Células T/diagnóstico por imagem , Linfoma de Células T/mortalidade , Linfoma Cutâneo de Células T/diagnóstico por imagem , Linfoma Cutâneo de Células T/mortalidade , Masculino , Pessoa de Meia-Idade , Paniculite/diagnóstico por imagem , Paniculite/mortalidade , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Estudos Retrospectivos , Medição de Risco , Estudos de Amostragem , Neoplasias Cutâneas/diagnóstico por imagem , Neoplasias Cutâneas/mortalidade , Espanha , Análise de Sobrevida , Adulto JovemRESUMO
Agminated melanocytic nevus is an uncommon type of mole, characterized by a local group of macular or papular pigmented lesions, well demarcated, without a common pigmented background. This pattern has also been associated with Spitz nevi, dysplastic melanocytic nevi, and non-melanocytic lesions.We describe the onset of an acquired agminated melanocytic nevus after dabrafenib treatment. Our case highlights paradoxical MAPK activation in the setting of single-agent BRAF blockade and underscores the importance of characterizing the diverse side effects of selective BRAF inhibitors. This is the first case, to our knowledge, of agminated melanocytic nevus in association with dabrafenib.
Assuntos
Antineoplásicos/efeitos adversos , Imidazóis/efeitos adversos , Neoplasias Pulmonares/tratamento farmacológico , Melanoma/tratamento farmacológico , Nevo Pigmentado/induzido quimicamente , Oximas/efeitos adversos , Neoplasias Cutâneas/patologia , Adulto , Evolução Fatal , Humanos , Neoplasias Pulmonares/secundário , Linfonodos/patologia , Masculino , Melanoma/secundário , Nevo Pigmentado/patologiaRESUMO
BACKGROUND: Kaposi's sarcoma (KS) in renal transplant recipients (RTRs) probably arises from a complex interplay of multiple factors. OBJECTIVE: In order to analyze the prevalence of KS in patients transplanted at the Cruces Hospital in Bilbao, together with their clinical features, treatment, and etiologic factors, we performed a study using the registry of RTRs in our center. METHODS: The records of 1,230 kidney transplant patients at the Cruces Hospital between 1979 and 1998 were reviewed. Immunosuppressive therapy was reduced once a diagnosis of KS was made. A nested polymerase chain reaction was used to detect human herpesvirus 8 (HHV-8) DNA in the biopsy tissue. The DNA was extracted from fresh tissue (n = 2) or from formalin-fixed, paraffin-embedded specimens (n = 5). RESULTS: Six cases of KS were diagnosed. All patients with cutaneous KS improved with a reduction in immunosuppressive drugs. HHV-8 was detected in 100% (2/2) of the frozen biopsies and 20% (1/5) of the formalin-fixed samples investigated. CONCLUSIONS: Our experience indicates that a continuous state of immunodeficiency is important for the development of KS in RTRs. The association, previously described between HHV-8 and transplant-associated KS, also exists in the studied population.
Assuntos
DNA Viral/análise , Falência Renal Crônica/epidemiologia , Falência Renal Crônica/cirurgia , Transplante de Rim/estatística & dados numéricos , Sarcoma de Kaposi/epidemiologia , Neoplasias Cutâneas/epidemiologia , Idoso , Biópsia por Agulha , Comorbidade , Feminino , Rejeição de Enxerto , Sobrevivência de Enxerto , Herpesvirus Humano 6/isolamento & purificação , Herpesvirus Humano 8/isolamento & purificação , Hospitais Urbanos , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Reação em Cadeia da Polimerase , Prevalência , Prognóstico , Sistema de Registros , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Sarcoma de Kaposi/diagnóstico , Sarcoma de Kaposi/tratamento farmacológico , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/tratamento farmacológico , Espanha/epidemiologiaRESUMO
Un varón de 11 años, con colestasis intrahepática marcada desde el período neonatal, presentaba un granuloma anular perforante (GAP) generalizado por tronco y extremidades. Aunque el GAP puede asociarse a diversas enfermedades, no tenemos noticias de que se haya publicado su asociación a colestasis intrahepática. El intenso prurito de la colestasis y el rascado subsiguiente podrían tener algún papel en el desarrollo del GAP (AU)
