RESUMO
After a history of intermittent vomiting, endoscopic biopsies of stomach and duodenum were collected from a 13-yr-old male snow leopard (Uncia uncia). On microscopic examination, monomorphic small lymphocytes expanded the duodenal mucosa and occasionally formed intraepithelial nests. Immunohistochemistry of the infiltrating small lymphocytes in the mucosa and within the epithelium had strong, perimembranous labeling for CD3e, with few CD79a-positive lymphocytes located at the base of the villi. Polymerase chain reaction (PCR) for antigen receptor rearrangements (PARR) of feline T-cell receptor gamma (TCRG) detected a monoclonal cell population. The sequence of the PCR product was 100% homologous with the feline TCRG gene. By histology, immunophenotyping, and PARR testing, a final diagnosis of enteropathy-associated T-cell lymphoma, small cell type, was made. Homology in the nucleotide sequence between U. uncia and the domestic cat (Felis catus) indicates that feline PARR testing for TCRG may be diagnostic in snow leopards.
Assuntos
Linfoma de Células T Associado a Enteropatia/veterinária , Felidae , Doenças Inflamatórias Intestinais/veterinária , Animais , Antineoplásicos Alquilantes/uso terapêutico , Antineoplásicos Hormonais/uso terapêutico , Clorambucila/uso terapêutico , Linfoma de Células T Associado a Enteropatia/diagnóstico , Linfoma de Células T Associado a Enteropatia/tratamento farmacológico , Linfoma de Células T Associado a Enteropatia/patologia , Doenças Inflamatórias Intestinais/diagnóstico , Masculino , Prednisolona/uso terapêutico , Vitamina B 12/administração & dosagem , Vitamina B 12/uso terapêutico , Complexo Vitamínico B/uso terapêuticoAssuntos
Antígenos CD20/biossíntese , Antineoplásicos Imunológicos/uso terapêutico , Linfoma de Células T Associado a Enteropatia/patologia , Recidiva Local de Neoplasia/patologia , Rituximab/uso terapêutico , Idoso , Biomarcadores Tumorais/análise , Linfoma de Células T Associado a Enteropatia/tratamento farmacológico , Linfoma de Células T Associado a Enteropatia/metabolismo , Evolução Fatal , Feminino , Humanos , Recidiva Local de Neoplasia/tratamento farmacológico , Recidiva Local de Neoplasia/metabolismoRESUMO
Enteropathy-associated T-cell lymphoma is a rare neoplasm with uniformly aggressive features that arises from intestinal T-cells. There is strong evidence supporting its association as a dire complication of celiac disease. The clinical presentation can vary from malabsorption and abdominal pain to an acute abdominal emergency. Originally, it was divided into types I and II in World Health Organization (WHO) classification schemes, reflective of epidemiology and differences in clinicopathologic features. The debate over the degree of separation of the two types is ongoing as new data emerges regarding the pathogenetics. The low incidence and variable patient factors are major barriers in conducting clinical trials and establishing standard treatment regimens. Yet, the collective experience demonstrates favorable outcomes with combination chemotherapy followed by an autologous hematopoietic stem cell transplant in patients who can tolerate such treatment. The prognosis remains dismal; thus, future research studies are warranted to identify effective novel therapies that can improve outcomes in this rare disease entity.
Assuntos
Linfoma de Células T Associado a Enteropatia/patologia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Doença Celíaca/complicações , Doença Celíaca/genética , Doença Celíaca/patologia , Aberrações Cromossômicas , Ciclofosfamida/uso terapêutico , Dieta Livre de Glúten , Doxorrubicina/uso terapêutico , Linfoma de Células T Associado a Enteropatia/complicações , Linfoma de Células T Associado a Enteropatia/tratamento farmacológico , Linfoma de Células T Associado a Enteropatia/genética , Antígenos HLA-DQ/genética , Humanos , Imunofenotipagem , Prednisona/uso terapêutico , Prognóstico , Vincristina/uso terapêuticoRESUMO
Enteropathy associated T-cell lymphoma (EATL) is a rare complication of celiac disease (CD). We report a case of EATL associated with CD revealed by acute intestinal obstruction. A North African woman of 38 years old with a history of infertility and chronic abdominal pain was admitted in emergency with acute intestinal obstruction. During the surgery, we found a tumor on the small intestine with mesenteric lymphadenopathy. Histology and immunohistochemistry of the specimen objectified a digestive T lymphoma CD3+ and immunological assessment of celiac disease was positive. The diagnosis of EATL was thus retained. Chemotherapy (CHOEP protocol) was established as well as gluten-free diet with a complete response to treatment. The EATL is a rare complication of CD that can be revealed by intestinal obstruction. The prognosis can be improved by early treatment involving surgery and chemotherapy. Its prevention requires early diagnosis of celiac and gluten-free diets.
Assuntos
Doença Celíaca/complicações , Linfoma de Células T Associado a Enteropatia/diagnóstico , Obstrução Intestinal/etiologia , Dor Abdominal/etiologia , Doença Aguda , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Doença Celíaca/terapia , Ciclofosfamida/uso terapêutico , Dieta Livre de Glúten , Doxorrubicina/uso terapêutico , Linfoma de Células T Associado a Enteropatia/tratamento farmacológico , Linfoma de Células T Associado a Enteropatia/etiologia , Etoposídeo/uso terapêutico , Feminino , Humanos , Prednisolona/uso terapêutico , Vincristina/uso terapêuticoRESUMO
AIM: To report the outcome of surgery in patients with (pre)malignant conditions of celiac disease (CD) and the impact on survival. METHODS: A total of 40 patients with (pre)malignant conditions of CD, ulcerative jejunitis (n = 5) and enteropathy associated T-cell lymphoma (EATL) (n = 35), who underwent surgery between 2002 and 2013 were retrospectively evaluated. Data on indications, operative procedure, post-operative morbidity and mortality, adjuvant therapy and overall survival (OS) were collected. Eleven patients with EATL who underwent chemotherapy without resection were included as a control group for survival analysis. Patients were followed-up every three months during the first year and at 6-mo intervals thereafter. RESULTS: Mean age at resection was 62 years. The majority of patients (63%) underwent elective laparotomy. Functional stenosis (n = 13) and perforation (n = 12) were the major indications for surgery. In 70% of patients radical resection was performed. Early postoperative complications, mainly due to leakage or sepsis, occurred in 14/40 (35%) of patients. Eight patients required reoperation. More patients who underwent resection in the acute setting (n = 3, 20%) died compared to patients treated in the elective setting. With a median follow-up of 20 mo, seven patients (18%) required reoperation due to long-term complications. Significantly more patients who underwent acute surgery could not be treated with adjuvant chemotherapy. Patients who first underwent surgical resection showed significantly better OS than patients who received chemotherapy without resection. CONCLUSION: Although the complication rate is high, the preferred first step of treatment in (pre)malignant CD consists of local resection as early as possible to improve survival.
Assuntos
Doença Celíaca/cirurgia , Procedimentos Cirúrgicos do Sistema Digestório , Linfoma de Células T Associado a Enteropatia/cirurgia , Neoplasias Intestinais/cirurgia , Lesões Pré-Cancerosas/cirurgia , Idoso , Antineoplásicos/uso terapêutico , Doença Celíaca/diagnóstico , Doença Celíaca/tratamento farmacológico , Doença Celíaca/mortalidade , Quimioterapia Adjuvante , Procedimentos Cirúrgicos do Sistema Digestório/efeitos adversos , Procedimentos Cirúrgicos do Sistema Digestório/mortalidade , Procedimentos Cirúrgicos Eletivos , Linfoma de Células T Associado a Enteropatia/diagnóstico , Linfoma de Células T Associado a Enteropatia/tratamento farmacológico , Linfoma de Células T Associado a Enteropatia/mortalidade , Feminino , Humanos , Neoplasias Intestinais/diagnóstico , Neoplasias Intestinais/tratamento farmacológico , Neoplasias Intestinais/mortalidade , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Países Baixos , Complicações Pós-Operatórias/etiologia , Lesões Pré-Cancerosas/diagnóstico , Lesões Pré-Cancerosas/tratamento farmacológico , Lesões Pré-Cancerosas/mortalidade , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Resultado do TratamentoRESUMO
BACKGROUND: Chidamide is a novel benzamide type of subtype-selective histone deacetylase (HDAC) inhibitor with unique mechanisms of action compared with marketed HDAC inhibitors. This phase II study was to evaluate the efficacy and safety of chidamide in relapsed or refractory peripheral T-cell lymphoma (PTCL) in Chinese population. PATIENTS AND METHODS: Patients with relapsed or refractory PTCL of different subtypes received chidamide of 30 mg orally twice per week. The primary end point was overall response rate (ORR). Responding patients should be confirmed at least 4 weeks after the criteria of the response were first met, and were reviewed by an independent review committee. RESULTS: Eighty-three patients were enrolled and 79 patients with eligible PTCL histology were for efficacy assessments. Patients enrolled over 10% were with subtypes of PTCL not otherwise specified (34%), anaplastic large-cell lymphoma (22%), extranodal natural killer (NK)/T-cell lymphoma, nasal type (20%), or angioimmunoblastic T-cell lymphoma (AITL, 13%). The ORR was 28% (22 of 79) including 14% (11 of 79) with complete response/unconfirmed complete response (CR/CRu). Median progression-free survival and overall survival were 2.1 and 21.4 months, respectively. AITL patients tended to have higher ORR (50%) and CR/CRu rate (40%), as well as more durable responses, to chidamide treatment. Most adverse events (AEs) were grade 1 or 2, and AEs ≥grade 3 that occurred in ≥10% patients were thrombocytopenia (22%), leucopenia (13%) and neutropenia (11%), respectively. CONCLUSION: Chidamide represents a novel oral benzamide class of HDAC inhibitor with significant single-agent activity and manageable toxicity in relapsed or refractory PTCL, and provides a much needed treatment option in this indication in China. Results led to China Food and Drug Administration approval of chidamide in this indication.
Assuntos
Aminopiridinas/uso terapêutico , Antineoplásicos/uso terapêutico , Benzamidas/uso terapêutico , Inibidores de Histona Desacetilases/uso terapêutico , Linfoma de Células T Periférico/tratamento farmacológico , Recidiva Local de Neoplasia/tratamento farmacológico , Adulto , Idoso , Intervalo Livre de Doença , Linfoma de Células T Associado a Enteropatia/tratamento farmacológico , Feminino , Humanos , Leucopenia/induzido quimicamente , Linfoma Extranodal de Células T-NK/tratamento farmacológico , Linfoma Anaplásico de Células Grandes/tratamento farmacológico , Linfoma Cutâneo de Células T/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Micose Fungoide/tratamento farmacológico , Neutropenia/induzido quimicamente , Neoplasias Cutâneas/tratamento farmacológico , Trombocitopenia/induzido quimicamente , Resultado do Tratamento , Adulto JovemRESUMO
Enteropathy-associated T-cell lymphoma (EATL) is a rare but potentially fatal cause of diarrhoea and weight loss. EATL commonly presents with abdominal pain, diarrhoea and weight loss, but can also present with complications such as bowel obstruction and perforation. It is a tumour of intraepithelial lymphocytes that occurs in a relatively young population. It is the most common neoplastic complication of coeliac disease, but can occur with no prior diagnosis of coeliac disease. This case demonstrates the difficulties that can be faced in diagnosing this disorder, particularly when there is no preceding history of coeliac disease. Early diagnosis is of utmost importance in order to start treatment before the effects of malnutrition increase the risk of complications from chemotherapy. Hence awareness of the condition among general medical physicians, to whom it will often present first, is essential. However, even with prompt diagnosis, outcomes for this condition remain poor.
Assuntos
Linfoma de Células T Associado a Enteropatia/complicações , Perfuração Intestinal/etiologia , Linfoma não Hodgkin/complicações , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Ascite/etiologia , Diarreia/etiologia , Linfoma de Células T Associado a Enteropatia/tratamento farmacológico , Linfoma de Células T Associado a Enteropatia/patologia , Evolução Fatal , Humanos , Intestino Delgado , Linfoma não Hodgkin/tratamento farmacológico , Linfoma não Hodgkin/patologia , Masculino , Pessoa de Meia-Idade , Redução de PesoAssuntos
Doença Celíaca/diagnóstico , Neoplasias Duodenais/diagnóstico , Linfoma de Células T Associado a Enteropatia/diagnóstico , Neoplasias Gástricas/diagnóstico , Sudorese , Redução de Peso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biópsia , Doença Celíaca/complicações , Neoplasias Duodenais/tratamento farmacológico , Neoplasias Duodenais/etiologia , Endoscopia do Sistema Digestório , Linfoma de Células T Associado a Enteropatia/complicações , Linfoma de Células T Associado a Enteropatia/tratamento farmacológico , Feminino , Humanos , Imuno-Histoquímica , Pessoa de Meia-Idade , Neoplasias Gástricas/tratamento farmacológico , Neoplasias Gástricas/etiologiaAssuntos
Linfoma de Células T Associado a Enteropatia , Glucocorticoides/administração & dosagem , Obstrução Intestinal , Doenças do Jejuno , Jejuno/patologia , Dor Abdominal , Administração Intravenosa , Idoso , Endoscopia do Sistema Digestório/métodos , Linfoma de Células T Associado a Enteropatia/complicações , Linfoma de Células T Associado a Enteropatia/diagnóstico , Linfoma de Células T Associado a Enteropatia/tratamento farmacológico , Linfoma de Células T Associado a Enteropatia/fisiopatologia , Feminino , Humanos , Obstrução Intestinal/diagnóstico , Obstrução Intestinal/etiologia , Obstrução Intestinal/fisiopatologia , Doenças do Jejuno/diagnóstico , Doenças do Jejuno/etiologia , Doenças do Jejuno/fisiopatologia , Terapêutica , Tomografia Computadorizada por Raios X/métodos , Vômito , Redução de PesoRESUMO
Enteropathy-associated T-cell lymphoma (EATL), an intestinal tumor of intraepithelial T lymphocytes, is a rare and highly aggressive disease. We herein describe a case of type II EATL with massive pyoid ascites in which a histological examination could not be performed despite emergency laparotomy that was successfully diagnosed using flow cytometry and the cell block technique to analyze the celomic fluid. This case suggests that EATL should be included in the differential diagnosis of pyoid ascites of unknown origin and that flow cytometry and the cell block technique of assessing celomic fluid are useful procedures for diagnosing EATL, especially in cases in which conducting a histological examination is impossible.
Assuntos
Ascite/patologia , Linfoma de Células T Associado a Enteropatia/diagnóstico , Citometria de Fluxo , Segunda Neoplasia Primária/diagnóstico , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Ascite/diagnóstico , Carcinoma de Células Renais/cirurgia , Ciclofosfamida/administração & dosagem , Doxorrubicina/administração & dosagem , Doxorrubicina/análogos & derivados , Emergências , Linfoma de Células T Associado a Enteropatia/tratamento farmacológico , Linfoma de Células T Associado a Enteropatia/patologia , Evolução Fatal , Feminino , Humanos , Perfuração Intestinal/etiologia , Neoplasias Renais/cirurgia , Laparotomia , Insuficiência de Múltiplos Órgãos/etiologia , Segunda Neoplasia Primária/tratamento farmacológico , Segunda Neoplasia Primária/patologia , Nefrectomia , Omento/patologia , Derrame Pleural/etiologia , Derrame Pleural/terapia , Prednisolona/administração & dosagem , Respiração Artificial , Insuficiência Respiratória/etiologia , Insuficiência Respiratória/terapia , Vincristina/administração & dosagemRESUMO
Enteropathy-associated T-cell lymphoma (EATL) is a rare disease that accounts for not more than 1.4% of all lymphomas. It is most common in Europe, followed by North America and Asia. The disease is associated with gluten-sensitive celiac disease in 50% of cases and divided into types I and II. Mean-dose CHOP-like therapy is ineffective, with a median overall survival of 7-10 months. With high-dose therapy, 5-year survival rates can be 60%, but it can be used in not more than half of the cases. This is associated with the serious somatic status of most patients at diagnosis and with a median age of 57-64 years. The article presents a literature review and a case of successful therapy in a 58-year-old patient with type I EATL using the mNHL-BFM-90 protocol and autologous hematopoietic stem cell transplantation.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Linfoma de Células T Associado a Enteropatia/terapia , Transplante de Células-Tronco Hematopoéticas , Linfoma de Células T Periférico/terapia , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Terapia Combinada , Relação Dose-Resposta a Droga , Linfoma de Células T Associado a Enteropatia/tratamento farmacológico , Humanos , Linfoma de Células T Periférico/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Transplante Autólogo , Resultado do TratamentoAssuntos
Enteroscopia de Duplo Balão , Linfoma de Células T Associado a Enteropatia/diagnóstico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Endoscopia por Cápsula , Linfoma de Células T Associado a Enteropatia/tratamento farmacológico , Humanos , Masculino , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios XRESUMO
Enteropathy-associated T-cell lymphoma (EATL) is a rare aggressive lymphoma that confers a poor prognosis with current treatment strategies. Given the rarity of this disease, prospective randomized trials are limited, and thus a standard validated treatment strategy is lacking. This report presents the disease course of a patient with EATL who was treated with single-agent brentuximab vedotin, an anti-CD30 conjugated antibody.
Assuntos
Linfoma de Células T Associado a Enteropatia/tratamento farmacológico , Linfoma de Células T Associado a Enteropatia/imunologia , Imunoconjugados/uso terapêutico , Antígeno Ki-1/imunologia , Anticorpos/imunologia , Brentuximab Vedotin , Humanos , Masculino , Pessoa de Meia-IdadeAssuntos
Endoscopia por Cápsula/métodos , Linfoma de Células T Associado a Enteropatia/diagnóstico , Linfoma de Células T Associado a Enteropatia/tratamento farmacológico , Intestino Delgado/metabolismo , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biópsia , Cisplatino/uso terapêutico , Ciclofosfamida/uso terapêutico , Citarabina/uso terapêutico , Doxorrubicina/uso terapêutico , Resistencia a Medicamentos Antineoplásicos , Etoposídeo/uso terapêutico , Humanos , Intestino Delgado/patologia , Linfonodos/patologia , Masculino , Oncologia/métodos , Prednisolona/uso terapêutico , Prednisona/uso terapêutico , Indução de Remissão , Terapia de Salvação/métodos , Vincristina/uso terapêuticoRESUMO
INTRODUCTION: Enteropathy-associated T-cell lymphoma is a rare form of T-cell lymphoma associated with a poor prognosis and the relative ineffectiveness of standard chemotherapy. The occurrence of haemophagocytic lymphohistiocytosis has been reported only once with this entity. PATIENTS AND METHODS: A retrospective study of 15 patients with enteropathy-associated T-cell lymphoma (type 1 in 12), followed-up in our units, since 1985. Two patients died before starting chemotherapy. The remaining 13 patients were treated with standard chemotherapy (n=7) and purine nucleotide analogues (n=6). RESULTS: Median follow-up was 8.7 (1-97) months. Surgery was required in 10 patients (66%) for intestinal complications (n=7) or elective small bowel resection (n=3). Survival probability was 40% and 20% at 1 and 5 years, respectively (Kaplan-Meier method). Survival was not significantly different between the two chemotherapy regimens. However, a slight decrease of febrile neutropenia was observed in the purine nucleotide analogues group (p=0.06). Haemophagocytic lymphohistiocytosis occurred in 6/15 (40%) cases. In these six patients, haemophagocytic lymphohistiocytosis was always fatal within 3 months. CONCLUSION: Enteropathy-associated T-cell lymphoma is associated with a poor outcome, independently of the chemotherapy regimens administered and frequent occurrence of haemophagocytic lymphohistiocytosis. The latter complication should be considered for urgent rescue therapy.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Linfoma de Células T Associado a Enteropatia/tratamento farmacológico , Linfo-Histiocitose Hemofagocítica/tratamento farmacológico , Nucleotídeos de Purina/uso terapêutico , Adulto , Idoso , Idoso de 80 Anos ou mais , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Cladribina/administração & dosagem , Progressão da Doença , Intervalo Livre de Doença , Linfoma de Células T Associado a Enteropatia/complicações , Linfoma de Células T Associado a Enteropatia/cirurgia , Feminino , Humanos , Estimativa de Kaplan-Meier , Linfo-Histiocitose Hemofagocítica/complicações , Linfo-Histiocitose Hemofagocítica/cirurgia , Masculino , Pessoa de Meia-Idade , Neutropenia/etiologia , Nucleotídeos de Purina/efeitos adversos , Estudos RetrospectivosRESUMO
A 65-year-old woman presented with a 6-month history of abdominal pain and watery diarrhea. Type II enteropathy-associated T-cell lymphoma (EATL) was diagnosed based on the clinical presentation and pathological examination of the tumor. The patient received combination chemotherapy but did not achieve remission. Subsequently, high-dose therapy (HDT) and autologous stem cell transplantation (ASCT) were performed. After these therapies, she achieved complete remission, which has been sustained for 18 months. Although the role of HDT-ASCT for EATL is still controversial, the clinical course of this patient suggests that ASCT can improve the prognosis in some patients with EATL.