RESUMO
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. METHODS: We analysed CTCL patients treated with BV from the Spanish Primary Cutaneous Lymphoma Registry (RELCP). RESULTS: Sixty-seven patients were included. There were 26 females and the mean age at diagnosis was 59 years. Forty-eight were mycosis fungoides (MF), 7 Sézary syndrome (SS) and 12 CD30+ lymphoproliferative disorders (CD30 LPD). Mean follow-up was 18 months. Thirty patients (45%) showed at least 10% of CD30+ cells among the total lymphocytic infiltrate. The median number of BV infusions received was 7. The overall response rate (ORR) was 67% (63% in MF, 71% in SS and 84% in CD30 LPD). Ten of 14 patients with folliculotropic MF (FMF) achieved complete or partial response (ORR 71%). The median time to response was 2.8 months. During follow-up, 36 cases (54%) experienced cutaneous relapse or progression. The median progression free survival (PFS) was 10.3 months. The most frequent adverse event was peripheral neuropathy (PN) (57%), in most patients (85%), grades 1 or 2. CONCLUSIONS: These results confirm the efficacy and safety of BV in patients with advanced-stage MF, and CD30 LPD. In addition, patients with FMF and SS also showed a favourable response. Our data suggest that BV retreatment is effective in a proportion of cases.
Assuntos
Imunoconjugados , Linfoma Cutâneo de Células T , Transtornos Linfoproliferativos , Micose Fungoide , Síndrome de Sézary , Neoplasias Cutâneas , Feminino , Humanos , Pessoa de Meia-Idade , Brentuximab Vedotin/uso terapêutico , Imunoconjugados/efeitos adversos , Neoplasias Cutâneas/patologia , Micose Fungoide/patologia , Síndrome de Sézary/patologia , Sistema de Registros , Antígeno Ki-1RESUMO
BACKGROUND: 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. METHODS: Clinical features, European Organization for Research and Treatment of Cancer/International Society for Cutaneous Lymphomas stage, light chain restriction, clonality, treatments, skin relapses, DFS, stage progression, extracutaneous disease, and outcome are analyzed in a series of 137 patients. RESULTS: Patients were classified as solitary lesion (T1) (n = 70; 51%), regional skin involvement (T2) (n = 40; 29%), and generalized skin lesions (T3) (n = 27; 20%). Surgical excision, local radiotherapy, or a combination were the initial treatment in 118 patients (86%). In 121 of 137 patients (88%) a complete remission was observed after initial treatment, including 99 of 106 patients (93%) with solitary or localized disease and 22 of 31 patients (71%) with multifocal lesions. Cutaneous relapses were observed in 53 patients (44%). Median DFS was 47 months. Patients with multifocal lesions or T3 disease showed higher relapse rate and shorter DFS. No significant differences were observed between surgery and radiotherapy, but surgery alone was associated with more recurrences at initial site. Overall survival at 5 and 10 years was 93%. Six patients (4%) developed extracutaneous disease during follow-up. LIMITATIONS: This was a case series retrospective study. CONCLUSION: Our results support long-term follow-up in patients with primary cutaneous marginal zone B-cell lymphomas. Disseminated skin lesions have higher relapse rate and shorter DFS suggesting further investigation on systemic therapies in such a group of patients.
Assuntos
Linfoma de Zona Marginal Tipo Células B/patologia , Linfoma de Zona Marginal Tipo Células B/terapia , Recidiva Local de Neoplasia/patologia , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/terapia , Adulto , Idoso , Idoso de 80 Anos ou mais , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Terapia Combinada , Intervalo Livre de Doença , Feminino , Seguimentos , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Radioterapia , Indução de Remissão , Estudos Retrospectivos , Taxa de Sobrevida , Adulto JovemRESUMO
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. METHODS: We report two cases with typical clinicopathological and immunophenotypical features of leg type lymphoma occurring simultaneously with a testicular B-cell lymphoma. We also report an additional case of PCDLBCL, leg type with secondary testicular involvement. RESULTS: All cases presented with typical red tumors exclusively located on the legs. Histologically, all cases showed a diffuse nonepidermotropic infiltrate composed of large blastic cells mainly centroblastic type. Phenotype showed strong positivity for Bcl-2, MUM-1, and FOXP1. Epstein-Barr virus stains and CD30 were negative in the 3 cases. In all cases the testicular infiltration showed the same pathological and phenotypical changes to those observed in the skin. LIMITATIONS: This was a retrospective case series study. CONCLUSION: Skin involvement by testicular B-cell lymphomas and PCDLBCL, leg type are indistinguishable on the basis of pathologic and immunophenotypical features, therefore specific investigation and clinic correlation are needed.
Assuntos
Linfoma de Células B/patologia , Linfoma Difuso de Grandes Células B/patologia , Neoplasias Cutâneas/patologia , Neoplasias Testiculares/secundário , Idoso , Humanos , Imunofenotipagem , Perna (Membro) , Linfoma de Células B/genética , Linfoma de Células B/imunologia , Linfoma Difuso de Grandes Células B/genética , Linfoma Difuso de Grandes Células B/imunologia , Masculino , Neoplasias Cutâneas/genética , Neoplasias Cutâneas/imunologiaRESUMO
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. No associated symptoms were present and the eruption regressed spontaneously after a variable period of days to months. Annually recurring erythema annulare centrifugum seems to represent a rare distinct clinical entity that has received little attention in literature. Clinicopathologic features of this peculiar clinical disorder and the differential diagnosis with other recurrent seasonal eruptions are reviewed.
Assuntos
Eritema/patologia , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva , Estações do AnoRESUMO
Epithelioid 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. In addition to a specific lymphomatous infiltrate, prominent dermal and/or subcutaneous granulomatous infiltrates were observed. Sarcoid-like granulomas were observed in two patients (one of them presented a granuloma annulare-like pattern in early lesions), granulomatous panniculitis was noted in one patient and in one patient with AILD, masses of epithelioid cells were noted. The clinicopathological features of cutaneous involvement by PTCL showing a florid epithelioid and/or granulomatous cell reaction are reviewed. Various histopathological patterns can be observed. The diagnostic difficulties of these cases are stressed.
Assuntos
Células Epitelioides/patologia , Granuloma/complicações , Linfoma de Células T Periférico/complicações , Dermatopatias/complicações , Idoso , Feminino , Granuloma/patologia , Humanos , Linfoma de Células T Periférico/patologia , Masculino , Pessoa de Meia-Idade , Pele/patologia , Dermatopatias/patologiaAssuntos
Hibridização Genômica Comparativa , Perfilação da Expressão Gênica , Linfoma Anaplásico de Células Grandes/genética , Neoplasias Cutâneas/genética , Regulação Neoplásica da Expressão Gênica , Humanos , Antígeno Ki-1/metabolismo , Linfoma Anaplásico de Células Grandes/metabolismo , Análise de Sequência com Séries de Oligonucleotídeos , Neoplasias Cutâneas/metabolismoRESUMO
El bexaroteno es un análogo de retinoide sintético caracterizado por unirse selectivamente a un tipo de receptores retinoides (receptores X) que fue diseñado para el tratamiento, entre otros, de estadios avanzados de linfoma cutáneo de células T resistentes a tratamientos convencionales. Se ha descrito que hasta el 40% de los pacientes que reciben bexaroteno pueden desarrollar hipotiroidismo secundario y hasta el 70%, hiperlipemia mixta grave, efecto común a otros retinoides. Presentamos a 3 pacientes que desarrollaron hipotiroidismo central y dislipemia tras el inicio de tratamiento con bexaroteno; en uno de ellos pudimos observar que estas alteraciones remitían tras la supresión del tratamiento (AU)
Bexarotene is a synthetic retinoid analogue that joints selectively to retinoid X receptor and has been designed for treatment of advanced stages of cutaneous T- cell lymphoma. Up to a 40% of patients treated with bexarotene develop central hypothyroidism while severe mixed dyslipidemia may be present in up to 70%. We report 3 patients that developed central hypothyroidism and dyslipidemia after bexarotene treatment was initiated. In one of them, we observed normalization of the thyroid function when bexarotene treatment was stopped (AU)
Assuntos
Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Humanos , Antineoplásicos/efeitos adversos , Tetra-Hidronaftalenos/efeitos adversos , Hipotireoidismo/induzido quimicamente , Hiperlipidemias/induzido quimicamente , Linfoma Cutâneo de Células T/tratamento farmacológico , Antineoplásicos/uso terapêutico , Tetra-Hidronaftalenos/uso terapêutico , Síndrome de Sézary/tratamento farmacológicoRESUMO
No disponible