RESUMO
To address the lack of contemporary population-based epidemiological studies of hepatosplenic T-cell lymphoma (HSTCL), we undertook a population-based study of ICD-O-3-coded HSTCL in England. We used the National Cancer Registration Dataset and linked datasets on hospital admissions, Systemic Anti-Cancer Therapy, socio-demographics, comorbidities and death, identifying cases from 1 January 2013 to 31 December 2019 with survival data up to 5 January 2021. Crude and directly age-standardised incidence rates per million persons per year were calculated. Crude and adjusted incidence rate ratios compared incidence between groups using Poisson regression. A Cox proportional hazards model estimated mortality risks adjusted for age, sex, ethnicity, deprivation and allogenic stem cell transplant (allo-SCT; time varying). We identified 44 patients, mean age 42 years. Median survival was 11 months, and 1 and 5 year survivals were 48% (95% CI 29%-43%) and 22% (95% CI 12%-42%) respectively. The age-standardised incidence was 0.1 per million/year. Incidence was higher in areas with greater deprivation (0.15 per million/year), and more cases than expected were in non-White patients (39%). Non-Whites had a twofold increased risk of death (adjusted hazard ratio 2.21 [95% CI 1.03-4.78]) even after adjusting for deprivation, younger age and allo-SCT. In conclusion, ethnicity and socio-economic status affect both the incidence and survival of HSTCL.
Assuntos
Linfoma de Células T , Neoplasias Esplênicas , Humanos , Masculino , Feminino , Incidência , Adulto , Pessoa de Meia-Idade , Linfoma de Células T/mortalidade , Linfoma de Células T/epidemiologia , Linfoma de Células T/terapia , Neoplasias Esplênicas/mortalidade , Neoplasias Esplênicas/epidemiologia , Idoso , Neoplasias Hepáticas/mortalidade , Neoplasias Hepáticas/epidemiologia , Neoplasias Hepáticas/terapia , Classe Social , Etnicidade/estatística & dados numéricos , Inglaterra/epidemiologia , Adulto Jovem , AdolescenteRESUMO
Haemangiosarcoma is a relatively common malignant tumour in dogs, and one of the primary outcomes of interest for the Golden Retriever Lifetime Study. This study collects longitudinal data and samples from a cohort of golden retrievers, with the aim of identification of nutritional, genetic, environmental, lifestyle and reproductive risk factors for cancers and other important diseases in dogs. This analysis describes the accumulating data and samples, which are available for use by researchers to fulfil the study's objectives. As of September 2022, 233/3044 dogs enrolled in the study had been diagnosed with haemangiosarcoma (7.65%), with an incidence rate of 1.10 cases per 100 dog-years. Visceral haemangiosarcoma was the most common, affecting 211/3044 study dogs (6.9%). One hundred and twenty eight visceral haemangiosarcoma diagnoses specified the presence of splenic tumours (60.7%) and 119 specified the presence of cardiac tumours (56.4%). The probability of remaining without a haemangiosarcoma diagnosis declined from 100% from approximately 4 years of age, to a 12 year probability of 91.1% in intact females (95% CI 84.4%-98.3%), 60.7% in neutered females (95% CI 41.6%-88.6%), 72.9% in intact males (95% CI 62.9%-84.6%) and 70.0% in neutered males (95% CI 53.4%-92.0%). The 1 year survival probability for visceral haemangiosarcoma was 1.42% (95% CI 0.37%-5.47%); for cutaneous haemangiosarcoma, it was 84.6% (95% CI 67.1%-99.99%). The accumulated data and samples are a considerable resource for further investigation of canine haemangiosarcoma and have a potential role in translational medicine.
Assuntos
Doenças do Cão , Neoplasias Cardíacas , Hemangiossarcoma , Neoplasias Cutâneas , Neoplasias Esplênicas , Humanos , Masculino , Feminino , Cães , Animais , Doenças do Cão/patologia , Hemangiossarcoma/epidemiologia , Hemangiossarcoma/veterinária , Neoplasias Cutâneas/veterinária , Neoplasias Cardíacas/veterinária , Neoplasias Esplênicas/epidemiologia , Neoplasias Esplênicas/veterináriaRESUMO
OBJECTIVE: To determine the prevalence of splenic malignancy in cats undergoing splenectomy and to investigate possible factors associated with post-operative outcome. ANIMALS: 62 client-owned cats that underwent splenectomy. METHODS: Medical records of 4 UK-based referral hospitals were searched and data reviewed retrospectively over 17 years. Factors associated with outcomes post-splenectomy were analyzed. RESULTS: 50 out of 62 cats (81%) were diagnosed with splenic neoplasia. Mast cell tumor ([MCT], 42%), hemangiosarcoma ([HSA], 40%), lymphoma and histiocytic sarcoma (6% each) were the most common tumor types. Fifteen cats (24%) presented with spontaneous hemoabdomen and were all diagnosed with splenic neoplasia. The diagnostic accuracy of cytology to detect splenic malignant lesions was 73% (100% for MCTs and 54% for mesenchymal tumors). Median survival time for cats with nonneoplastic splenic lesions was 715 days (IQR, 18 to 1,368) and 136 days for cats with splenic neoplasia (IQR, 35 to 348); median survival time was longer for cats with splenic MCT when compared to cats with HSA (348 vs 94 days; P < .001). Presence of metastatic disease and anemia (PCV < 24%) at diagnosis were associated with a poorer survival when considering all cats. Presence of anemia, a splenic mass on imaging or spontaneous hemoabdomen were associated with a diagnosis of HSA (P < .001). CLINICAL RELEVANCE: Benign splenic lesions were uncommon in this cohort of cats. Spontaneous hemoabdomen should prompt the clinician to suspect neoplasia in cats with splenic disease. Anemia and evidence of metastasis at diagnosis were poor prognostic factors regardless of the final diagnosis.
Assuntos
Anemia , Doenças do Gato , Doenças do Cão , Hemangiossarcoma , Neoplasias Esplênicas , Humanos , Gatos , Animais , Cães , Esplenectomia/efeitos adversos , Esplenectomia/veterinária , Estudos Retrospectivos , Prevalência , Neoplasias Esplênicas/epidemiologia , Neoplasias Esplênicas/cirurgia , Neoplasias Esplênicas/veterinária , Anemia/veterinária , Hemoperitônio/veterinária , Doenças do Cão/diagnóstico , Hemangiossarcoma/veterinária , Doenças do Gato/epidemiologiaRESUMO
Background: Hepatosplenic T-cell lymphoma (HSTCL) is a rare fatal T-cell neoplasm with unique clinical and laboratory features. There is, however, significant morphological and immunophenotypic heterogeneity which may lead to diagnostic dilemma. Aims and Objectives: The study was aimed to study the prevalence and clinic-pathological spectrum of this rare variant of T cell lymphoma in the Indian subcontinent. Material and Methods: A retrospective analysis of all consecutive cases of HSTCL diagnosed over a period of 6 years was carried out. The clinical and laboratory parameters of all these patient were reviewed and analysed. Results: A total of 12 cases of HSTCL were diagnosed during this period which accounted for 1.76% of all non-Hodgkin's lymphomas (NHLs) and 9.1% of all T-cell NHLs. The median (range) age of presentation was 23 (16-30) years.Leukocytosis, peripheral blood (PB) involvement, and a blastic morphology were noted in 41%, 67%, and 58% of the cases, respectively. FCI proved these cells to have a mature, dual-negative (CD4-/CD8-) T-cell phenotype with a gamma-delta T-cell receptor restriction. Frequent loss of CD5 expression (84%) was also noted. These patients invariably had a fatal outcome and majority died within a year of diagnosis. Conclusion: The incidence of leukocytosis and a blastoid morphology is quite frequent in HSTCL. Hence, a differential diagnosis of HSTCL should always be considered in young patients presenting with splenomegaly and exhibiting atypical lymphoid/blastoid cells in the PB or a marrow. An FCI can readily diagnose and differentiate them from an acute lymphoblastic leukemia/lymphoma.
Assuntos
Neoplasias Hepáticas , Linfoma de Células T , Neoplasias Esplênicas , Citometria de Fluxo , Humanos , Leucocitose , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/epidemiologia , Neoplasias Hepáticas/genética , Linfoma de Células T/diagnóstico , Linfoma de Células T/epidemiologia , Receptores de Antígenos de Linfócitos T gama-delta/análise , Receptores de Antígenos de Linfócitos T gama-delta/genética , Estudos Retrospectivos , Neoplasias Esplênicas/diagnóstico , Neoplasias Esplênicas/epidemiologiaRESUMO
Splenic marginal zone lymphoma (SMZL) is a rare lymphoproliferative disease involving B-cells and affecting elderly patients. SMZL plague peripheral blood and bone marrow, spleen. Lymph nodes are generally spared. SMZL is due to a protracted antigen stimulation of B lymphocytes and of microenvironment leading B-cell to polyclonal and then oligoclonal/monoclonal growth, promoting lymphoproliferation. Integration of the NOTCH2 and NFk-B signaling has been recently identified as the primary mechanism of neoplastic proliferation in SMZL. In total 20% of cases carry mutations in NOTCH2. Although SMZL has an indolent course, progression to diffuse large B-cell lymphoma occurs in about 10-15% of patients. Establishing the prognosis is a key step in disease management, depending on both individual risk and patients' health status. This review discusses tailored treatment of SMZL patients. Progression risk factors include nodal and extra-nodal involvement, peripheral lymphocytosis, anemia and thrombocytopenia. Patients with two or more score points have a median survival of <5 years. Watch and wait strategy is appropriate in low-risk and asymptomatic patients, whereas treatment of symptomatic patients ranges from splenectomy to rituximab monotherapy or associated with chemotherapy.
Assuntos
Antineoplásicos Imunológicos/uso terapêutico , Linfoma de Zona Marginal Tipo Células B/tratamento farmacológico , Linfoma de Zona Marginal Tipo Células B/patologia , Medicina de Precisão/métodos , Neoplasias Esplênicas/tratamento farmacológico , Neoplasias Esplênicas/patologia , Antineoplásicos/uso terapêutico , Progressão da Doença , Hepatite B/patologia , Hepatite C/epidemiologia , Hepatite C/patologia , Humanos , Linfoma de Zona Marginal Tipo Células B/epidemiologia , Linfoma de Zona Marginal Tipo Células B/cirurgia , Linfoma Difuso de Grandes Células B/fisiopatologia , NF-kappa B/metabolismo , Estadiamento de Neoplasias , Receptor Notch2/genética , Receptor Notch2/metabolismo , Receptores de Antígenos de Linfócitos B/genética , Receptores de Antígenos de Linfócitos B/imunologia , Medição de Risco , Fatores de Risco , Transdução de Sinais , Esplenectomia , Neoplasias Esplênicas/epidemiologia , Neoplasias Esplênicas/cirurgia , Microambiente Tumoral/fisiologiaRESUMO
We describe 36 patients with splenic marginal zone lymphoma (SMZL) with transformation (SMZL-T), including 15 from a series of 84 patients with SMZL diagnosed at the Hospital Clinic of Barcelona (HCB) and 21 diagnosed with SMZL-T in other centres. In the HCB cohort, the cumulative incidence of transformation at 5 years was 15%. Predictors for transformation were cytopenias, hypoalbuminaemia, complex karyotype (CK) and both the Intergruppo Italiano Linfomi (ILL) and simplified Haemoglobin, Platelet count, lactate dehydrogenase (LDH) and extrahilar Lymphadenopathy (HPLL)/ABC scores (P < 0·05). The only independent predictor for transformation in multivariate analysis was CK [hazard ratio (HR) 4·025, P = 0·05]. Patients with SMZL-T had a significantly higher risk of death than the remainder (HR 3·89, P < 0·001). Of the 36 patients with SMZL-T, one developed Hodgkin lymphoma and 35 a diffuse large B-cell lymphoma, 71% with a non-germinal centre phenotype. The main features were B symptoms, lymphadenopathy, and high serum LDH. CK was observed in 12/22 (55%) SMZL-T and fluorescence in situ hybridisation detected abnormalities of MYC proto-oncogene, basic helix-loop-helix transcription factor (MYC), B-cell leukaemia/lymphoma 2 (BCL2) and/or BCL6 in six of 14 (43%). In all, 21 patients received immunochemotherapy, six chemotherapy, one radiotherapy and three splenectomy. The complete response (CR) rate was 61% and the median survival from transformation was 4·92 years. Predictors for a worse survival in multivariate analysis were high-risk International Prognostic Index (HR 5·294, P = 0·016) and lack of CR (HR 2·67, P < 0·001).
Assuntos
Linfoma de Zona Marginal Tipo Células B/diagnóstico , Baço/patologia , Neoplasias Esplênicas/diagnóstico , Adulto , Idoso , Biomarcadores Tumorais , Transformação Celular Neoplásica , Análise Citogenética , Gerenciamento Clínico , Suscetibilidade a Doenças , Feminino , Humanos , Imuno-Histoquímica , Imunofenotipagem , Hibridização in Situ Fluorescente , Incidência , Linfoma de Zona Marginal Tipo Células B/epidemiologia , Linfoma de Zona Marginal Tipo Células B/etiologia , Linfoma de Zona Marginal Tipo Células B/metabolismo , Masculino , Pessoa de Meia-Idade , Gradação de Tumores , Estadiamento de Neoplasias , Prognóstico , Neoplasias Esplênicas/epidemiologia , Neoplasias Esplênicas/etiologia , Neoplasias Esplênicas/metabolismoRESUMO
INTRODUCTION: Treatment of Splenic (SMZL) and Nodal (NMZL) Marginal Zone Lymphoma is not consensual. Histologic transformation (HT) to aggressive lymphoma is a poorly understood event, with an unfavorable outcome. OBJECTIVES: Describe the clinical characteristics, treatment, outcomes and incidence of HT. METHODS: Characteristics of patients with SMZL and NMZL consecutively diagnosed in 8 Portuguese centers were retrospectively reviewed. Endpoints were overall survival (OS), time to first systemic treatment (TTFST), frequency of HT and time to transformation (TTT). RESULTS: This study included 122 SMZL and 68 NMZL, most of them received systemic treatment: 55.4% and 76.5%, respectively. Splenectomy was performed in 58.7% of patients with SMZL. Different treatment protocols were used. OS or TTFST did not differ significantly according to treatments. Given the small sample size, no conclusion can be made concerning the role of Rituximab in the treatment of NMZL and SMZL based in these results. HT was documented in 18 patients, mainly in SMZL, with a cumulative incidence at 5 years of 4.2%. We confirmed that age is a prognostic factor. CONCLUSION: Randomized prospective trials are needed to standardize treatment in MZL. Patients with HT did appear to have shorter OS in comparison with those who did not experience HT (OS 5 years of 68.4% vs. 80.4%), but the number of HT was too small to reach statistical significance.
Assuntos
Linfoma de Zona Marginal Tipo Células B/terapia , Neoplasias Esplênicas/terapia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Incidência , Linfoma de Zona Marginal Tipo Células B/epidemiologia , Masculino , Pessoa de Meia-Idade , Portugal , Estudos Prospectivos , Estudos Retrospectivos , Neoplasias Esplênicas/epidemiologia , Resultado do TratamentoRESUMO
Splenic marginal zone lymphoma (SMZL) is a rare low-grade B-cell lymphoma where associations with viral hepatitis and autoimmune and inflammatory diseases (AID) have been indicated. We aimed at assessing the prevalence of viral hepatitis and AID at SMZL diagnosis and outcome by treatment in a Swedish population-based study. A total of 277 SMZL patients registered in the Swedish Lymphoma Register in 2007-2017 were included. A history of viral hepatitis was reported in five (2%) patients and AID prior to SMZL in 72/240 (30%) patients. Treatment was given up front for 207 (75%) patients. Splenectomy with or without systemic treatment was performed in 119 (57%) and was associated with statistically significantly better overall survival [hazard ratio, HR = 0·47 (95% confidence interval, CI: 0·23-0·93), P = 0·03] and progression-free survival (HR = 0·55, 95% CI: 0·35-0·86, P = 0·008) compared to non-splenectomised patients in multivariable analyses. The up-front splenectomised group was younger and generally had a lower Ann Arbor stage, but also more frequently B symptoms and high lactate dehydrogenase than the non-splenectomised group. Viral hepatitis and AID history did not affect SMZL outcome. We report high incidence of AIDs and low incidence of viral hepatitis in this population-based study of SMZL. Splenectomy up front was associated with a favourable outcome.
Assuntos
Linfoma de Zona Marginal Tipo Células B/cirurgia , Esplenectomia , Neoplasias Esplênicas/cirurgia , Doenças Autoimunes/complicações , Feminino , Hepatite B/complicações , Hepatite C/complicações , Humanos , Linfoma de Zona Marginal Tipo Células B/epidemiologia , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Neoplasias Esplênicas/epidemiologia , Suécia/epidemiologia , Resultado do TratamentoRESUMO
Haemoperitoneum secondary to ruptured splenic tumours can be either benign or malignant in origin. The majority of previous studies of canine haemoperitoneum have been retrospective, which are associated with well-recognized biases, such as the potential to underappreciate the diversity of outcomes in a complex presentation such as haemoperitoneum. This study seeks to prospectively define perioperative morbidity and mortality of haemoperitoneum in dogs secondary to ruptured splenic masses. Forty dogs with haemoperitoneum secondary to a ruptured splenic mass met the inclusion criteria. As expected, the cohort predominately consisted of older large breed dogs. All dogs underwent preoperative staging and had a splenectomy performed. Histopathologic analysis was performed on the splenic mass, as well as any possible metastatic lesions that were noted intra-operatively. Perioperative care outside of splenectomy was delivered in specialty practices using current conventional approaches to care (eg, transfusions and anti-arrhythmic medications). Fifteen dogs (37.5%) had benign splenic tumours and were cured with surgery alone, whereas 62.5% had malignant disease (most often haemangiosarcoma [HSA]). Surgical outcomes were highly favourable in the vast majority of dogs. Indeed, 38 dogs (95%) survived and were discharged after a median hospitalization of 39.5 hours. Independent predictors of longer hospitalization times included receiving a transfusion and the development of an arrhythmia. Although small, this cohort defines distinctive and optimistic perspectives for dogs with haemoperitoneum from splenic tumour rupture. These favourable outcomes from this prospective study are sufficient to ask if larger prospective studies should be conducted to better inform owners during this challenging cancer emergency presentation.
Assuntos
Doenças do Cão/epidemiologia , Doenças do Cão/patologia , Hemangiossarcoma/veterinária , Neoplasias Esplênicas/veterinária , Ruptura Esplênica/veterinária , Animais , Doenças do Cão/cirurgia , Cães , Feminino , Hemangiossarcoma/epidemiologia , Hemangiossarcoma/patologia , Hemangiossarcoma/cirurgia , Hospitais Veterinários , Fígado/patologia , Masculino , Estudos Prospectivos , Esplenectomia/veterinária , Neoplasias Esplênicas/epidemiologia , Neoplasias Esplênicas/patologia , Neoplasias Esplênicas/cirurgia , Ruptura Esplênica/epidemiologia , Ruptura Esplênica/patologia , Ruptura Esplênica/cirurgia , Resultado do Tratamento , Estados Unidos/epidemiologiaRESUMO
BACKGROUND: Hepatosplenic T-cell lymphoma (HSTCL) is a rare, poorly treatable malignancy associated with therapy for IBD. Current knowledge of HSTCL risk in IBD comes from an era of step-up therapy, before earlier use of biologics or combination therapy was advocated to achieve deep mucosal healing. HSTCL risk among newer biologic classes has also not been evaluated. AIMS: To systematically characterise the association of HSTCL with biologic therapy for IBD. METHODS: We conducted a literature search and query of the Food and Drug Administration Adverse Event Reporting System to summarise HSTCL cases among IBD patients with prior biologic exposure. Demographics and immunosuppression exposure were extracted. Patients were stratified by current regimen (combination therapy, biologic monotherapy or no biologic), and biologic class (anti-TNF, anti-integrin, anti-interleukin 12/23). RESULTS: Sixty-two cases of HSTCL were identified from 2486 abstracts and 181 FDA Adverse Events Reporting System reports. The median age of affected patients was 28 years (range 12-81), and 83.6% were male, 84.7% had Crohn's disease. Five of 62 patients had no reported azathioprine/mercaptopurine exposure. Three patients within the cohort developed HSTCL after exposure to natalizumab, vedolizumab or ustekinumab; all three also had anti-TNF and azathioprine/mercaptopurine exposure. Forty-three of 49 (87.8%) patients with known outcomes died with a median survival of 5 months. CONCLUSIONS: Consistent with existing data, almost all identified HSTCL cases among IBD patients on biologic therapy had azathioprine/mercaptopurine exposure, and all cases on patients exposed to biologics had anti-TNF exposure. These data suggest initiating a patient-centred discussion before starting anti-TNF therapy or other biologics.
Assuntos
Produtos Biológicos/uso terapêutico , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/epidemiologia , Doenças Inflamatórias Intestinais/tratamento farmacológico , Doenças Inflamatórias Intestinais/epidemiologia , Neoplasias Hepáticas/epidemiologia , Linfoma de Células T/epidemiologia , Neoplasias Esplênicas/epidemiologia , Adolescente , Adulto , Sistemas de Notificação de Reações Adversas a Medicamentos/estatística & dados numéricos , Idoso , Idoso de 80 Anos ou mais , Criança , Feminino , Humanos , Neoplasias Hepáticas/induzido quimicamente , Linfoma de Células T/induzido quimicamente , Masculino , Pessoa de Meia-Idade , Neoplasias Esplênicas/induzido quimicamente , Estados Unidos/epidemiologia , United States Food and Drug Administration , Adulto JovemRESUMO
Cytology represents a useful diagnostic tool in the preliminary clinical approach to canine splenic lesions, and may prevent unnecessary splenectomy. However, few studies have evaluated diagnostic accuracy of cytology in the diagnosis of canine splenic neoplasms. The aim of this study was to determine overall accuracy, sensitivity, specificity, positive and negative predictive values (i.e. diagnostic accuracy indexes) of cytology for canine splenic neoplasms following Standards for the Reporting of Diagnostic Accuracy Studies (STARD) guidelines. A consecutive series of canine splenic cytological samples was retrospectively retrieved from the database of the Diagnostic Pathology Service of the Department of Veterinary Medicine (DIMEVET-University of Milan). Histopathology was set as the diagnostic reference standard. Cytological cases were enrolled when slides were available for review and when the same lesion was submitted for histopathology. Seventy-eight (78) lesions were included in the study. By histopathology, 56 were neoplastic and 22 were non-neoplastic. Cytology had an overall accuracy of 73.08% (95% C.I. 61.84%-82.50%), sensitivity of 64.29% (95% C.I. 50.36%-76.64%), specificity of 95.45% (95% C.I. 77.16%-99.88%), and positive and negative predictive values of 97.3% (95% C.I. 84.01%-99.60%) and 51.22% (95% C.I. 42.21%-60.15%), respectively. Low sensitivity and negative predictive value were balanced by very high specificity and positive predictive value. When positive for neoplasia, cytology represents a useful diagnostic tool to rule in splenic neoplasia, prompting surgery independently from other diagnostic tests. Conversely, a negative cytological result requires additional investigations to confirm the dog to be disease free.
Assuntos
Citodiagnóstico , Guias de Prática Clínica como Assunto , Neoplasias Esplênicas/diagnóstico , Neoplasias Esplênicas/veterinária , Animais , Intervalos de Confiança , Cães , Funções Verossimilhança , Prevalência , Neoplasias Esplênicas/epidemiologia , Neoplasias Esplênicas/patologiaRESUMO
Non-follicular small cell lymphomas include several entities whose clinical and pathological descriptions have been refined in the last 20 years. MALT lymphoma, developed at the expense of lymphoid tissue associated with the mucosa, is usually localized to a given organ, but can also disseminate. Some patients with MALT lymphoma can be treated by eradicating the associated infectious agent, whereas local treatment should be preferred for other cases ; disseminated forms and relapsed patients are eligible for anti-CD20 antibodies associated with cytotoxic agents. Patients with mantle cell lymphoma have benefited from many advances, including the use of cytarabine and bendamustine, anti-CD20 antibodies, intensive treatments (autograft) and recently targeted therapy (ibrutinib, inhibitor or the Bruton tyrosine kinase). Patients with splenic nodal marginal zone lymphomas should be evaluated for different options, of which immunochemotherapy remains important. For all these entities, the implementation of treatments may be delayed by several years for certain groups of patients. Although considered as incurable, the prognosis of these pathologies has improved significantly and the majority of patients will be able to live for many years with often treatment-free intervals.
Assuntos
Linfoma de Zona Marginal Tipo Células B , Fatores Etários , Idade de Início , Anticorpos Monoclonais/uso terapêutico , Diagnóstico Diferencial , Humanos , Imunoterapia/métodos , Imunoterapia/tendências , Linfoma de Zona Marginal Tipo Células B/classificação , Linfoma de Zona Marginal Tipo Células B/diagnóstico , Linfoma de Zona Marginal Tipo Células B/epidemiologia , Linfoma de Zona Marginal Tipo Células B/terapia , Terapia de Alvo Molecular/métodos , Terapia de Alvo Molecular/tendências , Recidiva Local de Neoplasia/epidemiologia , Recidiva Local de Neoplasia/terapia , Medicina de Precisão/métodos , Medicina de Precisão/tendências , Prognóstico , Neoplasias Esplênicas/diagnóstico , Neoplasias Esplênicas/epidemiologia , Neoplasias Esplênicas/patologia , Neoplasias Esplênicas/terapia , Neoplasias Gástricas/diagnóstico , Neoplasias Gástricas/epidemiologia , Neoplasias Gástricas/patologia , Neoplasias Gástricas/terapiaRESUMO
Various splenic diseases can result in a splenic mass and necessitate splenectomy. The objective of this study was to compare the prevalence of malignant and benign splenic diseases, and type of malignant disease, in dogs categorised by breed size. It was hypothesised that the prevalence of splenic disease would be significantly different in small versus large-breed dogs. All dogs had a splenic mass identified with ultrasonography or CT, and had a confirmed diagnosis. Dogs were categorised as small, medium and large breeds according to breed standards. There were 54 small, 139 medium and 41 large-breed dogs; 129/234, 55% (95% CI 49% to 61%) had malignant disease versus 105/234, 45% (95% CI 39% to 51%) with benign disease (P=0.117). The prevalence of malignant versus benign disease was not significantly different for small (P=0.276), medium (P=0.074) or large-breed dogs (P=0.080). Small-breed dogs were 2.3 times more likely than large-breed dogs to have benign disease. Small-breed dogs with malignant disease were one-third as likely as large-breed dogs to have haemangiosarcoma. In conclusion, the overall prevalence of malignant and benign diseases was 50:50; however, compared with large-breed dogs, small-breed dogs are more likely to have benign disease. When small dogs do have malignant disease, they are, however, less likely to have haemangiosarcoma. This information is important to consider in early discussions with owners of dogs of various breed sizes.
Assuntos
Tamanho Corporal , Doenças do Cão/epidemiologia , Hemangiossarcoma/veterinária , Esplenectomia/veterinária , Neoplasias Esplênicas/veterinária , Animais , Cruzamento , Doenças do Cão/diagnóstico , Cães , Feminino , Hemangiossarcoma/diagnóstico , Hemangiossarcoma/epidemiologia , Masculino , Prevalência , Esplenectomia/estatística & dados numéricos , Neoplasias Esplênicas/diagnóstico , Neoplasias Esplênicas/epidemiologia , Austrália Ocidental/epidemiologiaRESUMO
AIM: To evaluate the pattern of tumor relapse of pathological complete response (pCR) patients with locally advanced rectal cancer (LARC) following neoadjuvant chemoradiotherapy (nCRT) and total mesorectal excision (TME), and to identify predictive factors of distant metastasis in pCR patients after nCRT. METHOD: This was a retrospective analysis of 118 LARC patients who achieved a pCR following nCRT and TME from 2008 to 2015. Clinicopathological and therapeutic parameters were evaluated as possible predictors of distant metastasis-free survival (DMFS), and COX regression analysis was performed. RESULTS: After a median follow-up of 57 months, the 5-year overall and disease-free survival rates were 94.7% and 88.1%, respectively. Overall, 6 patients (5.1%) died, no local recurrence occurred, 13 patients (11%) developed distant metastases, including lung (nâ¯=â¯5), liver (nâ¯=â¯2), bone (nâ¯=â¯3), lung and brain (nâ¯=â¯1), peritoneal (nâ¯=â¯1), and spleen (nâ¯=â¯1) metastasis. On univariate analysis, tumor distance from the anal verge (HRâ¯=â¯0.706, Pâ¯=â¯0.039), acellular mucin pools (HRâ¯=â¯6.687, Pâ¯=â¯0.002), and MUC1 expression (HRâ¯=â¯8.280, Pâ¯<â¯0.001) were independently associated with DMFS. COX regression demonstrated that MUC1 expression (HRâ¯=â¯3.812, Pâ¯=â¯0.041) remained to be an independent predictor of DMFS in pCR patients. CONCLUSION: Distant metastasis still remained a major concern in pCR patients following nCRT and TME. Tumor distance from the anal verge, acellular mucin pools, and MUC1 expression were associated with distant metastasis in patients with pCR. MUC1 staining remained to be an independent risk factor for DMFS. Such information could facilitate treatment decision in these patients, such as adjuvant chemotherapy and follow-up.
Assuntos
Adenocarcinoma/terapia , Neoplasias Ósseas/epidemiologia , Neoplasias Hepáticas/epidemiologia , Neoplasias Pulmonares/epidemiologia , Neoplasias Retais/terapia , Adenocarcinoma/epidemiologia , Adenocarcinoma/secundário , Adulto , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Ósseas/secundário , Neoplasias Encefálicas/epidemiologia , Neoplasias Encefálicas/secundário , Capecitabina/administração & dosagem , Quimiorradioterapia , Quimioterapia Adjuvante , Intervalo Livre de Doença , Feminino , Fluoruracila/uso terapêutico , Humanos , Incidência , Leucovorina/uso terapêutico , Neoplasias Hepáticas/secundário , Neoplasias Pulmonares/secundário , Masculino , Mesentério/cirurgia , Pessoa de Meia-Idade , Mucina-1/metabolismo , Mucinas/metabolismo , Terapia Neoadjuvante , Metástase Neoplásica , Compostos Organoplatínicos/uso terapêutico , Oxaliplatina/administração & dosagem , Neoplasias Peritoneais/epidemiologia , Neoplasias Peritoneais/secundário , Protectomia , Modelos de Riscos Proporcionais , Neoplasias Retais/metabolismo , Neoplasias Retais/patologia , Indução de Remissão , Estudos Retrospectivos , Neoplasias Esplênicas/epidemiologia , Neoplasias Esplênicas/secundárioRESUMO
Angioedema due to acquired deficiency of the inhibitor of the first component of complement (C1-INH) is a rare disease known as acquired angioedema (AAE). About 70% of patients with AEE display autoantibodies to C1-INH, the remaining patients have no antibodies to C1-INH. The clinical features of C1-INH deficiency include recurrent, self-limiting local swellings involving the skin, the gastrointestinal tract, and the upper respiratory tract. Swelling is due to accumulation of bradykinin released from high molecular weight kininogen. Patients with angioedema due to acquired C1 inhibitor deficiency (AEE) often have an associated lymphoproliferative disease including Non-Hodgkin Lymphomas (NHL). Among AAE patients with NHL, splenic marginal zone lymphoma (SMZL) has a higher prevalence (66%) compared to general population (2%) In the present study, we focused on patients with SMZL in AAE. We found 24 AAE patients with NHL and, among them 15 SMZL (62.5% of all NHL). We found NOTCH 2 activation in 4 /15 patients (26.6%) with SMZL, while no patients carried MYD 88 or BIRC3 mutations. Restricted immunoglobulin gene repertoire analysis showed that the IGHV1-2*04 allele was found to be over-represented in the group of patients with or without lymphoproliferative disease presenting with autoantibodies to C1-INH (41 of 55 (75%) of patients; p value 0.011) when compared to the control group of patients with AEE without antibodies to C1-INH, (7 of 27 (26%) of patients). Immunophenotyping failed to demonstrate the presence of autoreactive clones against C1-inhibitor. Taken together, these findings suggest a role for antigenic stimulation in the pathogenesis of lymphomas associated with AEE.
Assuntos
Angioedema Hereditário Tipos I e II/complicações , Linfoma de Zona Marginal Tipo Células B/etiologia , Neoplasias Esplênicas/etiologia , Idoso , Idoso de 80 Anos ou mais , Proteína Inibidora do Complemento C1 , Feminino , Humanos , Linfoma de Zona Marginal Tipo Células B/epidemiologia , Masculino , Pessoa de Meia-Idade , Neoplasias Esplênicas/epidemiologiaRESUMO
Marginal zone lymphoma (MZL) is an uncommon indolent lymphoma classified into subtypes based on primary site of involvement: splenic, nodal and extranodal. MZLs' relative rarity has largely precluded adoption of a standard management strategy. Here, we provide an overview of the epidemiology, clinical behavior and therapeutic approaches for each subtype. Biologic insights into lymphomagenesis have identified B-cell receptor signaling as a rational therapeutic target. Recent clinical data suggest that novel agents targeting this pathway, including the Bruton's tyrosine kinase inhibitor, ibrutinib, show significant promise in treatment of relapsed MZL. More work is needed to evaluate these agents' activity in the front-line setting, possible combination regimens and the impact of resistance to B-cell receptor-targeted agents in order to optimize therapy in MZL.
Assuntos
Antineoplásicos/uso terapêutico , Linfoma de Zona Marginal Tipo Células B/tratamento farmacológico , Inibidores de Proteínas Quinases/uso terapêutico , Transdução de Sinais/efeitos dos fármacos , Neoplasias Esplênicas/tratamento farmacológico , Antineoplásicos/farmacologia , Resistencia a Medicamentos Antineoplásicos , Humanos , Linfonodos/patologia , Linfoma de Zona Marginal Tipo Células B/epidemiologia , Linfoma de Zona Marginal Tipo Células B/imunologia , Linfoma de Zona Marginal Tipo Células B/patologia , Recidiva Local de Neoplasia/prevenção & controle , Inibidores de Proteínas Quinases/farmacologia , Receptores de Antígenos de Linfócitos B/antagonistas & inibidores , Receptores de Antígenos de Linfócitos B/imunologia , Receptores de Antígenos de Linfócitos B/metabolismo , Transdução de Sinais/imunologia , Neoplasias Esplênicas/epidemiologia , Neoplasias Esplênicas/imunologia , Neoplasias Esplênicas/patologia , Resultado do TratamentoAssuntos
Subpopulações de Linfócitos B , Hepatite B , Hepatite C , Linfoma de Zona Marginal Tipo Células B , Neoplasias Esplênicas , Subpopulações de Linfócitos B/imunologia , Subpopulações de Linfócitos B/patologia , Feminino , Hepatite B/epidemiologia , Hepatite B/imunologia , Hepatite B/patologia , Hepatite C/epidemiologia , Hepatite C/imunologia , Hepatite C/patologia , Humanos , Linfoma de Zona Marginal Tipo Células B/epidemiologia , Linfoma de Zona Marginal Tipo Células B/imunologia , Linfoma de Zona Marginal Tipo Células B/patologia , Masculino , Prevalência , Neoplasias Esplênicas/epidemiologia , Neoplasias Esplênicas/imunologia , Neoplasias Esplênicas/patologiaAssuntos
Neoplasias Hepáticas/epidemiologia , Linfoma de Células T/epidemiologia , Neoplasias Esplênicas/epidemiologia , Adulto , Etnicidade/estatística & dados numéricos , Feminino , Humanos , Incidência , Renda , Cobertura do Seguro , Neoplasias Hepáticas/patologia , Neoplasias Hepáticas/terapia , Linfoma de Células T/patologia , Linfoma de Células T/terapia , Masculino , Morbidade/tendências , Prognóstico , Neoplasias Esplênicas/patologia , Neoplasias Esplênicas/terapia , Transplante de Células-Tronco/estatística & dados numéricos , Taxa de Sobrevida , Viagem , Estados Unidos/epidemiologiaRESUMO
Medical records of 396 dogs undergoing splenectomy for treatment of a splenic mass or nodular disease were reviewed retrospectively. Overall distribution of histopathologic diagnosis and clinicopathologic features were evaluated for 325 dogs that met inclusion criteria. Dogs were dichotomized into two groups based on weight, with the statistically derived cutoff identified as 27.8 kg. Malignancy was diagnosed in 58% of dogs, with no difference between small (55%) and large (61%) dogs (P = .291). Overall, 32% of splenic masses were hemangiosarcoma (HSA), which comprised 25 and 39% of all masses in small and large dogs, respectively. The diagnosis of HSA, non-HSA malignancy, or benign splenic disease was significantly different between the groups (P = .019). Of malignant diagnoses, HSA comprised 46 and 65% of small and large dog splenic neoplasms, respectively (P = .009). In both groups, dogs with HSA were significantly more likely to have preoperative anemia, hemoabdomen, thrombocytopenia, and a blood transfusion, as compared to dogs with non-HSA malignancy or benign lesions. Overall, dogs had similar odds of having a malignant splenic lesion regardless of weight, but dogs ≤27.8 kg were significantly less likely to be diagnosed with HSA.
Assuntos
Peso Corporal/fisiologia , Doenças do Cão/diagnóstico , Neoplasias Esplênicas/veterinária , Animais , Doenças do Cão/epidemiologia , Cães , Hemangiossarcoma/diagnóstico , Hemangiossarcoma/epidemiologia , Hemangiossarcoma/veterinária , Estudos Retrospectivos , Neoplasias Esplênicas/diagnóstico , Neoplasias Esplênicas/epidemiologiaRESUMO
OBJECTIVE To determine the frequency of malignancy and survival rates of dogs that underwent splenectomy for incidentally detected nonruptured splenic masses or nodules. DESIGN Retrospective case series. ANIMALS 105 client-owned dogs. PROCEDURES Medical records of dogs that underwent splenectomy at a veterinary teaching hospital between 2009 and 2013 were examined to identify patients with incidentally detected nonruptured splenic masses or nodules without associated hemoperitoneum. Only dogs with histologically confirmed diagnoses were included. Information regarding signalment, preoperative diagnostic tests, perioperative blood product transfusions, splenic mass diameter, histologic findings, adjunctive treatments, and survival time was collected and analyzed. RESULTS 74 of 105 (70.5%) patients had benign splenic lesions and 31 (29.5%) had malignant neoplasia, most commonly hemangiosarcoma (18/31 [58%]). The hazard of death decreased as preoperative PCV increased; histopathologic diagnosis of malignant neoplasia was significantly associated with an increased hazard of death. Median life expectancy of dogs with benign and malignant lesions was 436 and 110 days, respectively; 41 of 74 patients with benign lesions and 3 of 31 patients with malignant neoplasia were still alive at study conclusion. Median life expectancy of dogs with hemangiosarcoma was 132 days; only 7 of these 18 dogs received any adjunctive chemotherapeutic treatments. CONCLUSIONS AND CLINICAL RELEVANCE Incidentally found, nonruptured splenic masses or nodules without associated hemoperitoneum were most commonly benign. Results suggested that life expectancy for these dogs with incidentally detected benign or malignant splenic lesions that received prompt intervention was better than has previously been reported for other studied populations.