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1.
Am J Hematol ; 2024 Jul 03.
Artigo em Inglês | MEDLINE | ID: mdl-38957951

RESUMO

DISEASE OVERVIEW: Epstein Barr virus-positive (EBV+) diffuse large B-cell lymphoma (DLBCL), not otherwise specified (NOS) is an aggressive B-cell lymphoma associated with EBV infection included in the WHO classification of lymphoid neoplasms since 2016. Although historically associated to poor prognosis, outcomes seem to have improved in the era of chemoimmunotherapy. DIAGNOSIS: The diagnosis is established through meticulous pathological evaluation. Detection of EBV-encoded RNA (EBER) is the standard diagnostic method. The ICC 2022 specifies EBV+ DLBCL, NOS as occurring when >80% of malignant cells express EBER, whereas the WHO-HAEM5 emphasizes that the majority of tumor cells should be EBER positive without setting a defined threshold. The differential diagnosis includes plasmablastic lymphoma, DLBCL associated with chronic inflammation, primary effusion lymphoma, among others. RISK-STRATIFICATION: The International Prognostic Index (IPI) and the Oyama score can be used for risk-stratification. The Oyama score includes age >70 years and presence of B symptoms. The expression of CD30 and PD-1/PD-L1 are emerging as potential adverse but targetable biomarkers. MANAGEMENT: Patients with EBV+ DLBCL, NOS, should be staged and managed following similar guidelines than patients with EBV-negative DLBCL. EBV+ DLBCL, NOS, however, might have a worse prognosis than EBV-negative DLBCL in the era of chemoimmunotherapy. Therefore, inclusion of patients in clinical trials when available is recommended. There is an opportunity to study and develop targeted therapy in the management of patients with EBV+ DLBCL, NOS.

2.
Am J Hematol ; 99(8): 1586-1594, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38767403

RESUMO

DISEASE OVERVIEW: Plasmablastic lymphoma (PBL) is a rare CD20-negative aggressive lymphoma with a poor prognosis under standard treatment options. Though PBL is associated with human immunodeficiency virus infection and other immunosuppressed states, it can also affect immunocompetent individuals. DIAGNOSIS: The diagnosis requires a high clinical suspicion and pathological confirmation. EBER expression and MYC gene rearrangements are frequently detected. The differential diagnosis includes EBV+ diffuse large B-cell lymphoma, extracavitary primary effusion lymphoma, ALK+ DLBCL, and HHV8+ large B-cell lymphoma, among others. RISK STRATIFICATION: Age ≥60 years, advanced clinical stage, and high intermediate and high International Prognostic Index scores are associated with worse survival. MANAGEMENT: Combination chemotherapy regimens, such as EPOCH, are recommended. The addition of bortezomib, lenalidomide, or daratumumab might improve outcomes. Including PBL patients and their participation in prospective clinical trials is warranted.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica , Linfoma Plasmablástico , Humanos , Linfoma Plasmablástico/diagnóstico , Linfoma Plasmablástico/terapia , Linfoma Plasmablástico/tratamento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Medição de Risco , Ciclofosfamida/uso terapêutico , Doxorrubicina/uso terapêutico , Doxorrubicina/administração & dosagem , Vincristina/uso terapêutico , Vincristina/administração & dosagem , Prednisona/uso terapêutico , Lenalidomida/uso terapêutico , Lenalidomida/administração & dosagem , Prognóstico , Bortezomib/uso terapêutico , Bortezomib/administração & dosagem , Diagnóstico Diferencial , Gerenciamento Clínico , Pessoa de Meia-Idade , Linfoma Difuso de Grandes Células B/diagnóstico , Linfoma Difuso de Grandes Células B/terapia , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Linfoma Difuso de Grandes Células B/genética , Anticorpos Monoclonais , Etoposídeo
4.
Leuk Lymphoma ; 64(7): 1225-1233, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37132428

RESUMO

Readily accessible biomarkers for risk stratification in settings with limited resources are lacking. We evaluated the effect of high red distribution width-coefficient of variation (RDW-CV) values (>14%) on all-cause and lymphoma-specific mortality outcomes among 118 patients with peripheral T-cell lymphoma (PTCL) who received systemic treatment at two tertiary centers between 2010 and 2019. With a median follow-up of 45 months, patients with a high RDW-CV had a lower 4-year overall survival rate (34% vs. 45%, p = 0.015) and higher cumulative incidence of lymphoma mortality (54% vs. 34%, p = 0.007). RDW-CV >14% was associated with all-cause (adjusted Hazard Ratio [aHR] 1.98, 95% confidence interval [CI] 1.10-3.56) and lymphoma-specific mortality (aHR 2.64, 95% CI 1.32-5.29). In our study, RDW-CV emerges as an easily accessible and complementary prognostic biomarker for risk stratification among treated patients with de novo PTCL. Further research should validate the predictive role of RDW-CV in prospective cohorts.


Assuntos
Índices de Eritrócitos , Linfoma de Células T Periférico , Humanos , Prognóstico , Estudos Retrospectivos , Estudos Prospectivos , Linfoma de Células T Periférico/diagnóstico , Linfoma de Células T Periférico/terapia
6.
Cardiovasc Pathol ; 64: 107513, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36567046

RESUMO

Adult T-cell leukemia/lymphoma (ATLL) is an aggressive mature T-cell neoplasm caused by infection with the Human T-cell Lymphotropic Virus Type 1 (HTLV-1). Cardiac involvement in patients with ATLL is infrequent, and when it happens it is usually seen in aggressive ATLL subtypes. However, ATLL presenting as isolated cardiac valve involvement is extremely rare. To date, only three histologically proven cases of ATLL with isolated cardiac valve involvement have been reported. Herein, we describe a 61-year-old Peruvian man who presented heart failure symptoms secondary to progressive cardiac valve infiltration. The patient underwent mitral valve replacement with a mechanical prosthesis. Histopathological evaluation of the resected valve revealed leaflet thickening with a nodular appearance due to fibrous tissue containing atypical T-lymphocytes with Foxp3 expression, infiltrating all layers of the resected valve. Interestingly, tumor cells were distributed around an incidental venous malformation (i.e., cavernous hemangioma). Postoperative evaluation demonstrated positive serology for HTLV-1, and a diagnosis of ATLL was established. Postoperative positron emission tomography/computed tomography did not show lesions outside the heart and cell blood counts were within normal range with low level of circulating CD4+ CD25+ lymphoma cell counts (7%); therefore, patient's disease was considered as smoldering ATLL and a "watch and wait" strategy was pursued. Currently, the patient is alive with no progression of disease after 18 months from diagnosis. Isolated cardiac valve involvement by ATLL should be considered in the differential diagnosis of HTLV-1 carriers with progressive heart failure, even when systemic lymphoma involvement is absent or not apparent.


Assuntos
Vírus Linfotrópico T Tipo 1 Humano , Leucemia-Linfoma de Células T do Adulto , Linfoma , Adulto , Masculino , Humanos , Pessoa de Meia-Idade , Leucemia-Linfoma de Células T do Adulto/complicações , Leucemia-Linfoma de Células T do Adulto/cirurgia , Valvas Cardíacas/patologia
7.
Hematology ; 27(1): 1208-1216, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36326296

RESUMO

INTRODUCTION: Non-Hodgkin lymphomas (NHL) are the most frequently recognized entities among lymphoproliferative syndromes and rank fifth among neoplasms not associated with gender. There is scarce information on the clinical characteristics of the most frequent NHL, and no data on treatment regimens and their outcomes in Latin America. Although many factors affect a patient's possibilities of receiving treatment, the annual income per person/country is pivotal in Latin America. AIM: We present the clinical characteristics, risk groups, and treatment regimens of the three most frequent lymphoma subtypes in Latin America [diffuse large B-cell lymphoma (DLBCL), follicular lymphoma (FL), and peripheral T-cell lymphoma (PTCL)], based on the data collected by the largest study group of lymphoproliferative diseases in Latin America: The Latin American Study Group of Lymphoproliferative Disease [Grupo de Estudio de Linfoproliferativos de Latino America (GELL)]. OUTCOMES: The most frequent treatment regimen for B-cell lymphomas is immunochemotherapy (R-CHOP ≥70%), and CHOP for PTCL. Survival is similar to that reported by industrialized nations. We have no solid data on the results of treatment with salvage regimens nor stem cell transplantation in refractory/ relapsed NHL. CONCLUSION: In Latin America, the same treatment regimens are used as in highly developed countries, although we lack the necessary technology to apply CAR T-cell therapies or a network of trials sponsored by the pharmaceutical industry.


Assuntos
Linfoma Difuso de Grandes Células B , Linfoma de Células T Periférico , Humanos , América Latina/epidemiologia , Países em Desenvolvimento , Hispânico ou Latino
8.
JCO Glob Oncol ; 8: e2100380, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35939775

RESUMO

PURPOSE: Waldenstrom Macroglobulinemia (WM) is a rare lymphoma with distinct clinical features, and data from Latin American patients are lacking. Therefore, we aim to investigate the clinical, therapy, and outcome patterns of WM in Latin America. METHODS: We retrospectively analyzed patients with WM diagnosed between 1991 and 2019 from 24 centers in seven Latin American countries. The study outcomes were overall survival (OS) and progression-free survival (PFS). RESULTS: We identified 159 cases (median age 67 years, male 62%). Most patients (95%) were symptomatic at diagnosis. The International Prognostic Scoring System for WM (IPSSWM) at diagnosis was available in 141 (89%) patients (high-risk 40%, intermediate-risk 37%, and low-risk 23%). Twenty-seven (17%) patients were tested for MYD88L265P, with 89% (n = 24 of 27) carrying the mutation. First-line and second-line therapies were administered to 142 (89%) and 53 (33%) patients, respectively. Chemoimmunotherapy was the most commonly used first-line (66%) and second-line (45%) approach; only 18 (11%) patients received ibrutinib. With a median follow-up of 69 months, the 5-year OS rate was 81%. In treated patients, the 5-year OS and PFS rates were 78% and 59%, respectively. High-risk IPSSWM at treatment initiation was an independent risk factor for OS (adjusted hazard ratio: 4.73, 95% CI, 1.67 to 13.41, P = .003) and PFS (adjusted hazard ratio: 2.43, 95% CI, 1.31 to 4.50, P = .005). CONCLUSION: In Latin America, the management of WM is heterogeneous, with limited access to molecular testing and novel agents. However, outcomes were similar to those reported internationally. We validated the IPSSWM score as a prognostic factor for OS and PFS. There is an unmet need to improve access to recommended diagnostic approaches and therapies in Latin America.


Assuntos
Macroglobulinemia de Waldenstrom , Idoso , Humanos , América Latina/epidemiologia , Masculino , Mutação , Fator 88 de Diferenciação Mieloide/genética , Fator 88 de Diferenciação Mieloide/uso terapêutico , Estudos Retrospectivos , Macroglobulinemia de Waldenstrom/tratamento farmacológico , Macroglobulinemia de Waldenstrom/terapia
9.
Cancers (Basel) ; 14(9)2022 Apr 19.
Artigo em Inglês | MEDLINE | ID: mdl-35565181

RESUMO

Lymphomas are a highly heterogeneous group of hematological neoplasms. Given their ethiopathogenic complexity, their classification and management can become difficult tasks; therefore, new approaches are continuously being sought. Metabolic reprogramming at the lipid level is a hot topic in cancer research, and sphingolipidomics has gained particular focus in this area due to the bioactive nature of molecules such as sphingoid bases, sphingosine-1-phosphate, ceramides, sphingomyelin, cerebrosides, globosides, and gangliosides. Sphingolipid metabolism has become especially exciting because they are involved in virtually every cellular process through an extremely intricate metabolic web; in fact, no two sphingolipids share the same fate. Unsurprisingly, a disruption at this level is a recurrent mechanism in lymphomagenesis, dissemination, and chemoresistance, which means potential biomarkers and therapeutical targets might be hiding within these pathways. Many comprehensive reviews describing their role in cancer exist, but because most research has been conducted in solid malignancies, evidence in lymphomagenesis is somewhat limited. In this review, we summarize key aspects of sphingolipid biochemistry and discuss their known impact in cancer biology, with a particular focus on lymphomas and possible therapeutical strategies against them.

10.
Am J Hematol ; 97(7): 951-965, 2022 07.
Artigo em Inglês | MEDLINE | ID: mdl-35472248

RESUMO

DISEASE OVERVIEW: Epstein Barr virus-positive (EBV+) diffuse large B-cell lymphoma (DLBCL), not otherwise specified (NOS) is an entity included in the WHO classification of lymphoid neoplasms since 2016. EBV+ DLBCL, NOS, is an aggressive B-cell lymphoma associated with EBV infection, and a poor prognosis with standard chemotherapeutic approaches. DIAGNOSIS: The diagnosis is made through a careful pathological evaluation. Detection of EBV-encoded RNA (EBER) is considered standard for diagnosis; however, a clear cutoff for percentage of positive cells has not been defined. The differential diagnosis includes plasmablastic lymphoma (PBL), DLBCL associated with chronic inflammation, primary effusion lymphoma (PEL), among others. RISK-STRATIFICATION: The International Prognostic Index (IPI) and the Oyama score can be used for risk-stratification. The Oyama score includes age >70 years and presence of B symptoms. The expression of CD30 and PD-1/PD-L1 are emerging as potential adverse but targetable biomarkers. MANAGEMENT: Patients with EBV+ DLBCL, NOS, should be staged and managed following similar guidelines than patients with EBV-negative DLBCL. EBV+ DLBCL, NOS, however, might have a worse prognosis than EBV-negative DLBCL in the era of chemoimmunotherapy. Therefore, the inclusion of patients in clinical trials when available is recommended. There is an opportunity to study and develop targeted therapy in the management of patients with EBV+ DLBCL, NOS.


Assuntos
Infecções por Vírus Epstein-Barr , Linfoma Difuso de Grandes Células B , Idoso , Diagnóstico Diferencial , Infecções por Vírus Epstein-Barr/complicações , Infecções por Vírus Epstein-Barr/diagnóstico , Infecções por Vírus Epstein-Barr/patologia , Herpesvirus Humano 4 , Humanos , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Linfoma Difuso de Grandes Células B/terapia , Prognóstico
11.
Leuk Lymphoma ; 63(2): 315-325, 2022 02.
Artigo em Inglês | MEDLINE | ID: mdl-34585997

RESUMO

Adult T-cell leukemia/lymphoma (ATLL) is a largely incurable disease. Cutaneous involvement is common and could be first symptom of the disease. We analyzed 169 patients with ATLL of whom 63 had cutaneous involvement. Cutaneous involvement was found in 48, 27, 17, and 60% of acute, lymphomatous, chronic and smoldering ATLL cases, respectively. Eight cases had primary cutaneous tumoral variant. Erythroderma (24%) and plaques (22%) were the most frequent skin lesions. The presence of cutaneous involvement was associated with better overall survival compared to non-cutaneous involvement (aHR 0.55 [95% CI: 0.37-0.82], p < 0.01; 1-year OS 53 vs. 27%, respectively, p = 0.012). Combination zidovudine and interferon-alpha (AZT-IFN) yielded high response rates (overall response, OR = 100%, n = 8; complete response 62.5%) compared to chemotherapy (OR = 33.3%, n = 12/36). In conclusion, cutaneous involvement was associated with better survival in Latin American patients with ATLL. AZT-IFN demonstrated encouraging responses in ATLL patients with cutaneous involvement.


Assuntos
Vírus Linfotrópico T Tipo 1 Humano , Leucemia-Linfoma de Células T do Adulto , Linfoma , Neoplasias Cutâneas , Adulto , Humanos , Interferon-alfa/uso terapêutico , Leucemia-Linfoma de Células T do Adulto/diagnóstico , Leucemia-Linfoma de Células T do Adulto/tratamento farmacológico , Leucemia-Linfoma de Células T do Adulto/patologia , Linfoma/tratamento farmacológico , Estudos Retrospectivos , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/tratamento farmacológico
12.
JCO Glob Oncol ; 7: 1151-1166, 2021 07.
Artigo em Inglês | MEDLINE | ID: mdl-34270330

RESUMO

PURPOSE: Adult T-cell leukemia/lymphoma (ATLL) is an aggressive disease caused by the human T-cell leukemia virus type 1. Real-world data of ATLL in Latin America are lacking. PATIENTS AND METHODS: We analyzed patients with ATLL (acute, lymphomatous, chronic, and smoldering) encountered in 11 Latin American countries between 1995 and 2019. Treatment response was assessed according to the 2009 consensus report. Survival curves were estimated using the Kaplan-Meier method and log-rank test. RESULTS: We identified 253 patients; 226 (lymphomatous: n = 122, acute: n = 73, chronic: n = 26, and smoldering: n = 5) had sufficient data for analysis (median age 57 years). Most patients with ATLL were from Peru (63%), Chile (17%), Argentina (8%), and Colombia (7%). Hypercalcemia was positively associated with acute type (57% v lymphomatous 27%, P = .014). The median survival times (months) were 4.3, 7.9, 21.1, and not reached for acute, lymphomatous, chronic, and smoldering forms, with 4-year survival rates of 8%, 22%, 40%, and 80%, respectively. First-line zidovudine (AZT)-interferon alfa (IFN) resulted in an overall response rate of 63% (complete response [CR] 24%) for acute. First-line chemotherapy yielded an overall response rate of 41% (CR 29%) for lymphomatous. CR rate was 42% for etoposide, cyclophosphamide, vincristine, doxorubicin, and prednisone versus 12% for cyclophosphamide, vincristine, doxorubicin, and prednisone-like regimen (P < .001). Progression-free survival at 1 year for acute type patients treated with AZT-IFN was 67%, whereas 2-year progression-free survival in lymphomatous type patients who achieved CR after chemotherapy was 77%. CONCLUSION: This study confirms Latin American ATLL presents at a younger age and has a high incidence of lymphomatous type, low incidence of indolent subtypes, and worse survival rates as compared with Japanese patients. In aggressive ATLL, chemotherapy remains the preferred choice for lymphomatous favoring etoposide-based regimen (etoposide, cyclophosphamide, vincristine, doxorubicin, and prednisone), whereas AZT-IFN remains a good first-line option for acute subtype.


Assuntos
Leucemia-Linfoma de Células T do Adulto , Linfoma , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Argentina , Chile , Colômbia , Humanos , América Latina/epidemiologia , Leucemia-Linfoma de Células T do Adulto/tratamento farmacológico , Leucemia-Linfoma de Células T do Adulto/epidemiologia , Pessoa de Meia-Idade , Peru/epidemiologia
13.
Leuk Lymphoma ; 62(12): 2845-2853, 2021 12.
Artigo em Inglês | MEDLINE | ID: mdl-34151703

RESUMO

Anaplastic lymphoma kinase-positive (ALK+) large B-cell lymphoma (LBCL) is a rare CD20-negative aggressive lymphoma. Given its rarity, data on ALK + LBCL are scarce and limited to case reports and small case series. Our systematic review included 184 unique cases published in the literature and shows that ALK + LBCL can affect individuals at any age, has a male predominance and is not associated with chronic viral infections. The malignant cells express ALK, VS38c, BLIMP-1, EMA, c-MYC, and BOB-1. The STAT3/STAT5, PI3K/AKT, PLCG2, and ERK pathways are important in the pathophysiology of ALK + LBCL. The prognosis of ALK + LBCL is poor with a 5-year survival rate of 28%. Early disease stage is associated with better outcomes. ALK inhibitors and other targeted agents could be of value in the treatment of ALK + LBCL. Additional research is needed to better understand, diagnose and treat ALK + LBCL.


Assuntos
Linfoma Difuso de Grandes Células B , Linfoma Anaplásico de Células Grandes , Quinase do Linfoma Anaplásico , Humanos , Linfoma Difuso de Grandes Células B/diagnóstico , Linfoma Difuso de Grandes Células B/metabolismo , Linfoma Difuso de Grandes Células B/terapia , Masculino , Fosfatidilinositol 3-Quinases , Prognóstico , Receptores Proteína Tirosina Quinases/genética , Receptores Proteína Tirosina Quinases/metabolismo
14.
Neuropathology ; 41(1): 49-57, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32776398

RESUMO

Systemic embolization has been reported in up to 40% of patients with left atrial myxoma, half of them with cerebral involvement. However, development of intracerebral embolization associated with parenchymal seeding of the myxoma emboli is an extremely rare complication, with only 36 histologically diagnosed cases reported in the published literature. We describe a 69-year-old woman who arrived at the emergency service with hemiparesis associated with drug-resistant epilepsy and a medical history of resection of a left atrial myxoma 10 months previously. Cranial computed tomography revealed multiple large lesions of heterogeneous density and cystic components in the occipital lobes and posterior fossa parenchyma. Histopathological analyses after stereotactic biopsy of the occipital lesion revealed infiltrative myxoma cells with benign histological findings and uniform expression of calretinin similar to that of the primary cardiac myxoma. Additional immunohistochemical studies confirmed brain parenchymal seeding of the myxoma cells with strong expression of interleukin-6 (IL-6) and focal expression of matrix metalloproteinases-2 (MMP-2). Here, we discuss the clinicopathological features of intracerebral embolization of left atrial myxomas associated with progressive parenchymal seeding of the tumor emboli and the potential pathogenic role of IL-6 and MMPs.


Assuntos
Neoplasias Cardíacas/metabolismo , Interleucina-6/biossíntese , Embolia Intracraniana/metabolismo , Metaloproteinase 2 da Matriz/biossíntese , Mixoma/metabolismo , Inoculação de Neoplasia , Idoso , Feminino , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/cirurgia , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/cirurgia , Humanos , Embolia Intracraniana/diagnóstico por imagem , Embolia Intracraniana/cirurgia , Mixoma/diagnóstico por imagem , Mixoma/cirurgia
15.
Clin Lymphoma Myeloma Leuk ; 20(10): 637-646, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32513598

RESUMO

INTRODUCTION: We aimed at investigating the prognostic role of the neutrophil-to-lymphocyte ratio (NLR) in 2 independent cohorts of Latin American patients with diffuse large B-cell lymphoma (DLBCL) treated with chemoimmunotherapy. PATIENTS AND METHODS: The learning cohort was composed of 274 patients and the validation cohort of 323 patients, for a total of 597 patients. An optimal NLR cutoff ≥ 4 was determined using receiver operating characteristic analysis. RESULTS: In multivariate models, NLR ≥ 4 was independently associated with lower odds for complete response to chemoimmunotherapy in the learning (odds ratio, 0.46; P = .006) and the validation cohort (odds ratio, 0.49; P = .01), and independently associated with worse survival in the learning (hazard ratio, 1.55; P = .04) and the validation cohort (hazard ratio, 1.80; P = .003). CONCLUSIONS: The adverse prognostic value of NLR ≥ 4 was independent of the International Prognostic Index and the National Comprehensive Cancer Network-International Prognostic Index score. Based on the results of this multi-institutional study, NLR ≥ 4 emerges as an adverse prognostic factor in Latin American patients with DLBCL treated with chemoimmunotherapy.


Assuntos
Linfócitos/metabolismo , Linfoma Difuso de Grandes Células B/sangue , Neutrófilos/metabolismo , Feminino , Humanos , Linfoma Difuso de Grandes Células B/mortalidade , Masculino , Pessoa de Meia-Idade , Prognóstico , Análise de Sobrevida
16.
Rev. Fac. Med. Hum ; 20(2): 178-179, abr.- jun. 2020.
Artigo em Inglês, Espanhol | LILACS-Express | LILACS | ID: biblio-1120710

RESUMO

El Linfoma/Leucemia T del adulto es una entidad linfoproliferativa T agresiva asociado al retrovirus, HTLV-1. La infección por HTLV-1 es endémica en Japón, Caribe, África, Sudamérica y en el Medio Este. En Sudamérica, tenemos a Perú, Brasil, Colombia y Chile. El Perú es endémico para este virus. La prevalencia del retrovirus en Europa y USA es menor del 1% pero en Perú se estima alrededor del 3% de la población adulta sana es portadora del retrovirus. De Chile, existen varios reportes de ATLL desde el año 1992 por la Dra. Cabrera y col.


Adult T - lymphoma / leukemia is an aggressive T lymphoproliferative entity associated retrovirus HTLV-1- T. HTLV-1 infection is endemic in Japan, the Caribbean, Africa, South America, and the Middle East. In South America, we have Peru, Brazil, Colombia and Chile. Peru is endemic for this virus. The prevalence of retroviruses in Europe and the USA. USA It is less than 1% but in Peru it is estimated that around 3% of the healthy adult population is a carrier of the retrovirus. From Chile, there have been several ATLL reports since 1992 by Dra. Cabrera et al.

17.
Am J Hematol ; 95(4): 435-445, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-32072672

RESUMO

DISEASE OVERVIEW: Epstein Barr virus-positive (EBV+) diffuse large B-cell lymphoma (DLBCL), not otherwise specified (NOS) is an entity included in the 2016 WHO classification of lymphoid neoplasms. EBV+ DLBCL, NOS, is an aggressive B-cell lymphoma associated with chronic EBV infection, and a poor prognosis with standard chemotherapeutic approaches. DIAGNOSIS: The diagnosis is made through a careful pathological evaluation. Detection of EBV-encoded RNA (EBER) is considered standard for diagnosis; however, a clear cutoff for positivity has not been defined. The differential diagnosis includes plasmablastic lymphoma (PBL), DLBCL associated with chronic inflammation and primary effusion lymphoma (PEL), among others. RISK-STRATIFICATION: The International Prognostic Index (IPI) and the Oyama score can be used for risk-stratification. The Oyama score includes age >70 years and presence of B symptoms. The expression of CD30 and PD-1/PD-L1 are emerging as potential adverse but targetable biomarkers. MANAGEMENT: Patients with EBV+ DLBCL, NOS, should be staged and managed following similar guidelines than patients with EBV-negative DLBCL. EBV+ DLBCL, NOS, however, might have a worse prognosis than EBV-negative DLBCL in the era of chemoimmunotherapy. There is an opportunity to study and develop targeted therapy in the management of patients with EBV+ DLBCL, NOS.


Assuntos
Infecções por Vírus Epstein-Barr , Linfoma Difuso de Grandes Células B , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biomarcadores Tumorais , Terapia Combinada , Diagnóstico Diferencial , Gerenciamento Clínico , Infecções por Vírus Epstein-Barr/diagnóstico , Humanos , Inibidores de Checkpoint Imunológico/uso terapêutico , Imunoterapia , Imunoterapia Adotiva , Linfoma Difuso de Grandes Células B/diagnóstico , Linfoma Difuso de Grandes Células B/genética , Linfoma Difuso de Grandes Células B/terapia , Linfoma Difuso de Grandes Células B/virologia , Transtornos Linfoproliferativos/diagnóstico , Prognóstico , Inibidores de Proteassoma/uso terapêutico , Medição de Risco , Transdução de Sinais , Terapias em Estudo
18.
Clin Lymphoma Myeloma Leuk ; 19(9): e551-e557, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31320254

RESUMO

INTRODUCTION: The red blood cell distribution width (RDW) is an easy-to-obtain laboratory value that has emerged as a potential prognostic factor in solid and hematologic malignancies. PATIENTS AND METHODS: We evaluated 121 patients with de novo diffuse large B-cell lymphoma (DLBCL) treated with standard chemoimmunotherapy at our institution between 2010 and 2012. We categorized patients with high RDW (> 14.6%) and normal RDW (11.6%-14.6%). We fitted multivariate regression models for complete response (CR) and overall survival (OS). RESULTS: Patients with high RDW were less likely to achieve CR to chemoimmunotherapy than patients with normal RDW (48% vs. 83%; P < .001). The 5-year OS rate for patients with high RDW was lower than in patients with normal RDW (51% vs. 79%; P = .001). In multivariate regression models, high RDW was independently associated with lower odds of achieving CR (odds ratio, 0.32; 95% confidence interval [CI], 0.12-0.83; P = .02) and with higher risk of death from any cause (hazard ratio [HR], 2.04; 95% CI, 1.03-4.02; P = .04) than normal RDW in patients with DLBCL treated with chemoimmunotherapy. High RDW remained an independent adverse factor for OS after adjustment for the International Prognostic Index and the National Comprehensive Cancer Network-International Prognostic Index scores with HR 2.20 (95% CI, 1.12-4.31; P = .02) and HR 2.67 (95% CI 1.28-5.59; P = .009), respectively. CONCLUSION: High RDW appears to be an adverse predictive and prognostic factor in patients with de novo DLBCL treated with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone).


Assuntos
Índices de Eritrócitos , Linfoma Difuso de Grandes Células B/sangue , Linfoma Difuso de Grandes Células B/mortalidade , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biomarcadores , Ciclofosfamida/efeitos adversos , Ciclofosfamida/uso terapêutico , Doxorrubicina/efeitos adversos , Doxorrubicina/uso terapêutico , Feminino , Humanos , Estimativa de Kaplan-Meier , Linfoma Difuso de Grandes Células B/diagnóstico , Linfoma Difuso de Grandes Células B/terapia , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Razão de Chances , Prednisona/efeitos adversos , Prednisona/uso terapêutico , Prognóstico , Modelos de Riscos Proporcionais , Rituximab/efeitos adversos , Rituximab/uso terapêutico , Vincristina/efeitos adversos , Vincristina/uso terapêutico
19.
Rev. Fac. Med. Hum ; 19(2): 40-47, Apr-June. 2019. ilus, tab
Artigo em Espanhol | LILACS-Express | LILACS | ID: biblio-1136634

RESUMO

Objetivo: Evaluar el valor pronostico del índice linfocito monocito (ILM) en la sobrevida global de los pacientes con Linfoma de células grandes B difuso (LCGBD) del Hospital Edgardo Rebagliati Martins. Métodos: Estudio longitudinal retrospectivo. Se incluyó a los casos de LCGBD diagnosticados en el Hospital Nacional Edgardo Rebagliati Martins durante el período 2010-2017. No se realizó muestreo, se trabajó con la totalidad de la población que cumplió con los criterios de selección por ser esta pequeña y accesible. Se revisó las historias clínicas de los pacientes obteniéndose información del tiempo en meses de supervivencia, del ILM así como de variables sociodemográficas, clínicas y de laboratorio. Resultados: Se incluyó en el análisis a 121 pacientes con LCGBD; de ellos, el 57% eran de sexo femenino y 66.1% eran mayores de 60 años. De acuerdo al Status Zubrod, el 66,5% correspondieron al grado de mejor pronóstico y el 59.5% presento sintomas B asociados. Cerca del 60% fueron diagnosticados en estadios I y II y el 57% presento compromiso extraganglionar. El análisis bivariado con el modelo de riesgos proporcionales de Cox mostró que el ILM<2 constituyó un predictor de la supervivencia global del LCGBD (p=0,011) estimándose un HR=2.2 (IC 95%: 1.2-4.1); asimismo, un ILM< 1,7 también constituyó predictor (p=0,009) estimándose un HR=2.2 (IC 95%: 1.2-4.1). El ILM<2,7 no constituyó predictor de la supervivencia global. Conclusión: El ILM podría utilizarse como un índice pronóstico debido a que constituye un predictor de la supervivencia global de los pacientes con LCGBD del Hospital Nacional Edgardo Rebagliati Martins.


Objetive: To evaluate the prognostic value of the monocyte lymphocyte index (ILM) in the overall survival of patients with diffuse large B-cell lymphoma (LCGBD) of the Edgardo Rebagliati Martins Hospital. Methods: Retrospective longitudinal study. We included cases of LCGBD diagnosed in the National Hospital Edgardo Rebagliati Martins during the period 2010-2017. No sampling was done, we worked with the entire population that met the selection criteria because it is small and accessible. The patients' clinical histories were reviewed, obtaining information about the time in months of survival, ILM, as well as sociodemographic, clinical and laboratory variables. Results: 121 patients with LCGBD were included in the analysis; of them, 57% were female and 66.1% were older than 60 years. According to the Zubrod Status, 66.5% corresponded to the degree of better prognosis and 59.5% presented associated B symptoms. About 60% were diagnosed in stages I and II and 57% presented extranodal involvement. The bivariate analysis with the Cox proportional hazards model showed that the ILM <2 constituted a predictor of the overall survival of the LCGBD (p = 0.011), estimating HR = 2.2 (95% CI: 1.2-4.1); likewise, an ILM <1.7 was also a predictor (p = 0.009) with an estimated HR = 2.2 (95% CI: 1.2-4.1). ILM <2.7 was not a predictor of overall survival.. Conclusion: The ILM could be used as a prognostic index because it is a predictor of the overall survival of patients with LCGBD of the Edgardo Rebagliati Martins National Hospital.

20.
Rev. Fac. Med. Hum ; 19(2): 40-47, Apr-June. 2019.
Artigo em Inglês, Espanhol | LILACS-Express | LILACS | ID: biblio-1025831

RESUMO

Objetivo: Evaluar el valor pronostico del índice linfocito monocito (ILM) en la sobrevida global de los pacientes con Linfoma de células grandes B difuso (LCGBD) del Hospital Edgardo Rebagliati Martins. Métodos: Estudio longitudinal retrospectivo. Se incluyó a los casos de LCGBD diagnosticados en el Hospital Nacional Edgardo Rebagliati Martins durante el período 2010-2017. No se realizó muestreo, se trabajó con la totalidad de la población que cumplió con los criterios de selección por ser esta pequeña y accesible. Se revisó las historias clínicas de los pacientes obteniéndose información del tiempo en meses de supervivencia, del ILM así como de variables sociodemográficas, clínicas y de laboratorio. Resultados: Se incluyó en el análisis a 121 pacientes con LCGBD; de ellos, el 57% eran de sexo femenino y 66.1% eran mayores de 60 años. De acuerdo al Status Zubrod, el 66,5% correspondieron al grado de mejor pronóstico y el 59.5% presento sintomas B asociados. Cerca del 60% fueron diagnosticados en estadios I y II y el 57% presento compromiso extraganglionar. El análisis bivariado con el modelo de riesgos proporcionales de Cox mostró que el ILM<2 constituyó un predictor de la supervivencia global del LCGBD (p=0,011) estimándose un HR=2.2 (IC 95%: 1.2-4.1); asimismo, un ILM< 1,7 también constituyó predictor (p=0,009) estimándose un HR=2.2 (IC 95%: 1.2-4.1). El ILM<2,7 no constituyó predictor de la supervivencia global. Conclusión: El ILM podría utilizarse como un índice pronóstico debido a que constituye un predictor de la supervivencia global de los pacientes con LCGBD del Hospital Nacional Edgardo Rebagliati Martins.


Objetive: To evaluate the prognostic value of the monocyte lymphocyte index (ILM) in the overall survival of patients with diffuse large B-cell lymphoma (LCGBD) of the Edgardo Rebagliati Martins Hospital. Methods: Retrospective longitudinal study. We included cases of LCGBD diagnosed in the National Hospital Edgardo Rebagliati Martins during the period 2010-2017. No sampling was done, we worked with the entire population that met the selection criteria because it is small and accessible. The patients' clinical histories were reviewed, obtaining information about the time in months of survival, ILM, as well as sociodemographic, clinical and laboratory variables. Results: 121 patients with LCGBD were included in the analysis; of them, 57% were female and 66.1% were older than 60 years. According to the Zubrod Status, 66.5% corresponded to the degree of better prognosis and 59.5% presented associated B symptoms. About 60% were diagnosed in stages I and II and 57% presented extranodal involvement. The bivariate analysis with the Cox proportional hazards model showed that the ILM <2 constituted a predictor of the overall survival of the LCGBD (p = 0.011), estimating HR = 2.2 (95% CI: 1.2-4.1); likewise, an ILM <1.7 was also a predictor (p = 0.009) with an estimated HR = 2.2 (95% CI: 1.2-4.1). ILM <2.7 was not a predictor of overall survival.. Conclusion: The ILM could be used as a prognostic index because it is a predictor of the overall survival of patients with LCGBD of the Edgardo Rebagliati Martins National Hospital.

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