RESUMO
Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma, primary cutaneous CD30 lymphoproliferative disorders (pc CD30 LPD) being the second most prevalent. There is evidence that MF and pc CD30 LPD may coexist and share T-cell clonality, suggesting a common origin. These findings were supported by a T-cell receptor clonality assessment by the polymerase chain reaction coupled with capillary electrophoresis, although results produced by this method may be ambiguous. We describe an otherwise healthy 46-year-old man who developed, over the course of 5 months, a tumor consisting of primary cutaneous anaplastic large cell lymphoma and, subsequently, several papules of lymphomatoid papulosis (LyP). Both lymphomas appeared on a single patch of MF, which had been present on the patient's right buttock for at least 2 years. T-cell receptor clonality of the 3 types of neoplastic lesions and apparently non-involved skin were assessed by a next-generation sequencing-based method. We found that MF, primary cutaneous anaplastic large cell lymphoma and LyP harbored the same top 2 clones. Non-involved skin harbored other T-cell clones. In this patient, these findings suggest that MF, LyP and pc CD30 LPD were different clinicopathological manifestations arising from the neoplastic proliferation of the same T-cell clone.
Assuntos
Linfoma Anaplásico de Células Grandes/patologia , Papulose Linfomatoide/patologia , Micose Fungoide/patologia , Neoplasias Primárias Múltiplas/patologia , Neoplasias Cutâneas/patologia , Humanos , Antígeno Ki-1 , Masculino , Pessoa de Meia-Idade , Linfócitos T/patologiaAssuntos
Linfócitos T CD4-Positivos/patologia , Transtornos Linfoproliferativos/diagnóstico , Transtornos Linfoproliferativos/patologia , Dermatopatias/diagnóstico , Dermatopatias/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Glândulas Écrinas/patologia , Feminino , Folículo Piloso/patologia , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
BACKGROUND: The calcineurin pathway is often activated in mycosis fungoides. We aimed to assess the activity and safety of topical pimecrolimus, a calcineurin inhibitor, in patients with early mycosis fungoides. METHODS: PimTo-MF was a single-arm, multicentre, phase 2 trial done at six medical centres in Spain. Patients (aged ≥18 years) had histologically confirmed early mycosis fungoides (stages IA-IIA) and an Eastern Cooperative Oncology Group performance status of 0-1. Key exclusion criteria included the use of concurrent treatments for mycosis fungoides, including sunbathing, topical or systemic corticosteroids, and other calcineurin inhibitors. Patients applied topical pimecrolimus 1% cream on their skin lesions twice daily for 16 weeks (1 g per 2% of body surface), with subsequent follow-up of 12 months. Dosage modifications were not allowed. To evaluate adherence to the treatment, patients were instructed to return all empty tubes to the hospital (as per drug accountability protocols). The primary endpoint was the overall response ratein the intention-to-treat population. PimTo-MF is registered with EudraCT, 2014-001377-14, and is complete. FINDINGS: Between March 1, 2015, and Sept 30, 2016, 39 patients were enrolled. All patients were assessable, with a median age of 51·5 years (IQR 45-62), and the population was predominantly male (24 male [62%], 15 female [38%]). Median follow-up after baseline was 5·7 years (IQR 5·7-6·2). 22 (56%) of 39 patients had an overall response (one complete response, 21 partial responses). Responses were observed across IA (14 [54%] of 26 patients) and IB (eight [73%] of 11 patients) clinical stages, but not IIA. Topical pimecrolimus was well tolerated and no patient required a dose reduction or discontinued treatment because of unacceptable drug-related toxicity. No patients were lost to follow-up or discontinued treatment. 13 (33%) of 39 patients reported adverse events; transitory mild burning or pruritus (grade 1) was the most common, seen in eight (21%) patients. In three (8%) of these patients, the burning or pruritus was considered related to treatment. No grade 4 or 5 adverse events were observed. INTERPRETATION: Pimecrolimus 1% cream seems active and safe in patients with early stage mycosis fungoides. Our findings should be taken with caution until long-term follow-up data are obtained that confirm the safety of this treatment. Further controlled clinical trials are warranted to confirm these results. FUNDING: Instituto de Salud Carlos III and the European Regional Development Fund. TRANSLATION: For the Spanish translation of the abstract see Supplementary Materials section.
Assuntos
Micose Fungoide , Neoplasias Cutâneas , Adolescente , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Micose Fungoide/tratamento farmacológico , Prurido/tratamento farmacológico , Neoplasias Cutâneas/tratamento farmacológico , Tacrolimo/efeitos adversos , Tacrolimo/análogos & derivadosRESUMO
INTRODUCTION: Tocilizumab (TCZ) is an interleukin-6 receptor antagonist, which has been used for the treatment of severe SARS-CoV-2 pneumonia (SSP), which aims to ameliorate the cytokine release syndrome (CRS) induced acute respiratory distress syndrome (ARDS). However, there are no consistent data about who might benefit most from it. METHODS: We administered TCZ on a compassionate-use basis to patients with SSP who were hospitalized (excluding intensive care and intubated cases) and who required oxygen support to have a saturation >93%. The primary endpoint was intubation or death after 24 h of its administration. Patients received at least one dose of 400 mg intravenous TCZ from March 8, 2020 to April 20, 2020. RESULTS: A total of 207 patients were studied and 186 analyzed. The mean age was 65 years and 68% were male patients. A coexisting condition was present in 68% of cases. Prognostic factors of death were older age, higher IL-6, d-dimer and high-sensitivity C-reactive protein (HSCRP), lower total lymphocytes, and severe disease that requires additional oxygen support. The primary endpoint (intubation or death) was significantly worst (37% vs 13%, p < 0·001) in those receiving the drug when the oxygen support was high (FiO2 >0.5%). CONCLUSIONS: TCZ is well tolerated in patients with SSP, but it has a limited effect on the evolution of cases with high oxygen support needs.
Assuntos
Anticorpos Monoclonais Humanizados/administração & dosagem , Tratamento Farmacológico da COVID-19 , Adulto , Idoso , Idoso de 80 Anos ou mais , Proteína C-Reativa/imunologia , COVID-19/imunologia , COVID-19/mortalidade , COVID-19/virologia , Ensaios de Uso Compassivo , Cuidados Críticos/estatística & dados numéricos , Feminino , Humanos , Fatores Imunológicos , Interleucina-6/imunologia , Masculino , Pessoa de Meia-Idade , SARS-CoV-2/efeitos dos fármacos , SARS-CoV-2/fisiologia , EspanhaRESUMO
No disponible
Assuntos
Detecção Precoce de Câncer/métodos , Neoplasias do Colo do Útero/diagnóstico , Infecções por Papillomavirus/diagnóstico , Programas de Rastreamento/métodos , Testes Obrigatórios , Esfregaço Vaginal/métodos , Teste de Papanicolaou/métodos , Fatores Etários , Papillomaviridae/patogenicidade , Análise Custo-BenefícioRESUMO
No disponible