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1.
Zhongguo Shi Yan Xue Ye Xue Za Zhi ; 29(5): 1498-1503, 2021 Oct.
Artigo em Chinês | MEDLINE | ID: mdl-34627430

RESUMO

OBJECTIVE: To investigate the clinical characteristics and treatment outcome of patients with Burkitt lymphoma. METHODS: The clinical data of 27 patients with Burkitt Lymphoma were collected and retrospectively analyzed, the clinical characteristics, laboratory data, survival and the factors affecting the prognosis were also analyzed. RESULTS: Among the 27 patients (mainly for adults), the median age was 30 (15-83) years old, the ratio of male and female was 3.5∶1. There was no EB virus infection in all the patients, 92.6% of the patients showed extranodal organs involvement, 40.7% of them were leukemic stage, 85.2% patients belonged to Ⅲ and Ⅳ stage, 74.1% patients belonged to high/high-middle risk according to IPI index. In the terms of molecular biology, five patients were treated with next-generation sequencing test, and the MYC gene mutations were detected out in alt the patients, and the most common mutations were CCND3, ID3 and TP53. The overall response rate (ORR) for all the patients was 85.2%, the complete response (CR) rate was 63.0%, and the partial response rate was 22.2%, the 5-year progression-free survival rate and overall survival rate of the patients was 76.6% and 76.6%, respectively, which showed that the efficacy of the patients in high-dose methotrexate treatment group was higher than that in the non-high high-dose methotrexate treatment group. For the patients treated with LMB89 chemotherapy, the CR was 78.6%, ORR was 100%, the 5-year survival rate was 92.9%, which was superior to the patients treated with other regimens. Auto-hematopoietic stem cell transplantation as consolidation treatment could improve the prognosis for those patients who could not tolerate high-dose chemotherapy. Univariate analysis showed that ECOG score, the level of LDH>500 U/L, WBC level, CNS involvement, short-term effect and LMB89 regimen were the risk factors affecting the prognosis of the patients. CONCLUSION: The adult Burkitt lymphoma are highly aggressive. For the patients in high-dose methotrexate treatment group, especially LMB89 regimen can improve the survival of the patients, and to choose HSCT as a consolidation treatment can be a choice for those patients who could not tolerate high-dose chemotherapy.


Assuntos
Linfoma de Burkitt , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Protocolos de Quimioterapia Combinada Antineoplásica , Linfoma de Burkitt/tratamento farmacológico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Indução de Remissão , Estudos Retrospectivos , Adulto Jovem
2.
Blood Adv ; 5(17): 3322-3332, 2021 09 14.
Artigo em Inglês | MEDLINE | ID: mdl-34477813

RESUMO

IKZF1 deletions (ΔIKZF1) are commonly detected in B-precursor acute lymphoblastic leukemia (ALL; B-ALL) and are widely assumed to have a significant impact on outcome. We compared the ability of multiplex ligand-dependent probe amplification (MLPA) and polymerase chain reaction (PCR) to detect ΔIKZF1 and to determine the impact on event-free survival of patients with precursor B-ALL aged 23 to 65 years recruited to the completed trial UKALL14 (ISRCTN 66541317). From 655 recruits with BCR-ABL1+ and BCR-ABL1- B-ALL, all available diagnostic DNA samples (76% of the recruited population) were screened by multiplex end point PCR covering 4 deletions: dominant-negative (DN) Δ4-7 or the loss of function Δ2-7, Δ4-8, and Δ2-8 (n = 498), MLPA (n = 436), or by both (n = 420). Although patients with BCR-ABL1- ΔIKZF1 were more likely to have minimal residual disease at the end of induction, we did not find any impact of ΔIKZF1 (including subgroup analysis for DN or loss-of-function lesions) or the IKZF1plus genotype on event-free, overall survival, or relapse risk by univariable or multivariable analyses. Consistent with the technical approach, MLPA not only detected a wider range of deletions than PCR but also failed to detect some PCR-detected lesions. The main difference between our study and others reporting an association between ΔIKZF1 and outcome is the older age of participants in our population. The impact of ΔIKZF1 in ALL may be less marked in an older population of patients. Our study underscores the need for analyses in large, harmonized data sets. This trial was registered at www.clinicaltrials.gov as #NCT01085617.


Assuntos
Linfoma de Burkitt , Leucemia-Linfoma Linfoblástico de Células Precursoras , Adulto , Idoso , Proteínas de Fusão bcr-abl/genética , Humanos , Fator de Transcrição Ikaros/genética , Pessoa de Meia-Idade , Reação em Cadeia da Polimerase , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética
3.
J Pak Med Assoc ; 71(9): 2265-2267, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34580528

RESUMO

In this part of the world, Burkitt's lymphoma (BL) of mandible considers a unique entity, while BL involving the jaw is endemic in Africa. BL being one of the most speedily growing malignancy, can cause immense destruction in the maxillofacial region. This case report highlights a 3-year-old child affected with BL of mandible. The presenting complaint of the patient was a rapidly enlarging swelling on the right side of the face, causing disfigurement and interfering with the normal oral functions. Confirmatory diagnosis of BL was established after correlating the clinical, radiographic, and biopsy features. Later on a tumour board discussion was held for this case, and the child underwent chemotherapy. Unfortunately, he could not survive after the second cycle of chemotherapy. This emphasises that for every fast growing jaw lesion, BL should have a significant position in the list of differential diagnosis, even in this part of the world without considering the age of the patient.


Assuntos
Linfoma de Burkitt , Linfoma de Burkitt/diagnóstico , Linfoma de Burkitt/tratamento farmacológico , Pré-Escolar , Diagnóstico Diferencial , Grupos Étnicos , Humanos , Masculino , Mandíbula/diagnóstico por imagem , Paquistão
4.
Turk J Pediatr ; 63(4): 639-647, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34449146

RESUMO

BACKGROUND: Chemotherapy with high dose methotrexate is the mainstay of treatment for Burkitt lymphoma (BL), especially to manage central nervous system (CNS) disease. However, methotrexate administration requires close drug level monitoring for appropriate folinic acid rescue, which might not be readily available in all centers. In this study, we assessed the long-term treatment outcomes of a modified Non-Hodgkin lymphoma (NHL)-Berlin-Frankfurt-Munster (BFM) 90 regimen in pediatric high-risk BL without CNS involvement. METHODS: Between 1999 and 2011, 42 patients (median age: 7 years) with advanced-stage BL were treated with modified NHL-BFM 90 regimen (methotrexate at a dose of 1 g/m2). Demographic data, stage, lactate dehydrogenase (LDH) and treatment results were retrospectively evaluated. The patients were assessed for toxicity, survival and CNS recurrence. RESULTS: Thirty-six patients had Stage III and six had Stage IV disease, respectively. The median LDH level was 1,432 IU/L. Four patients died of infectious and metabolic complications. One patient had local recurrence at the 48 < sup > th < /sup > month of the follow-up and he is in the second remission for 72 months. In Kaplan-Meier analysis, the overall survival and event-free survival rates at 10 years were found as 90 % and 88 %, respectively. None of our patients died of treatment failure. CONCLUSIONS: The administration of the reduced dose of methotrexate seems to not compromise treatment success nor increase the risk of CNS recurrence in high-risk BL without CNS involvement. The limitation of the study is that it is not randomized. Our treatment scheme might be considered for centers without methotrexate measurement facility.


Assuntos
Linfoma de Burkitt , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Asparaginase , Linfoma de Burkitt/tratamento farmacológico , Criança , Daunorrubicina , Humanos , Masculino , Recidiva Local de Neoplasia , Prednisona , Estudos Retrospectivos , Resultado do Tratamento , Vincristina
5.
Arch. argent. pediatr ; 119(4): e330-e334, agosto 2021. ilus
Artigo em Espanhol | LILACS, BINACIS | ID: biblio-1281754

RESUMO

El linfoma es la neoplasia maligna de cabeza y cuello más común en la población pediátrica. Las anifestaciones clínicas de linfoma en amígdala palatina son generalmente asimetría amigdalina, alteración en la apariencia de la mucosa y adenopatías cervicales. Ante una hipertrofia amigdalina unilateral, se debe diferenciar de cuadros infecciosos, procesos crónicos, otros tumores o simplemente una hiperplasia amigdalina benigna. El seguimiento estricto de la asimetría amigdalina es un pilar importante a la hora del diagnóstico de los pacientes con linfoma amigdalino, debido a que un tratamiento temprano es fundamental para el pronóstico. Se presenta una niña de 14 años con hipertrofia amigdalina unilateral y odinofagia, de aproximadamente 21 días de evolución sin respuesta al tratamiento antibiótico, con diagnóstico de linfoma de Burkitt. Presenta remisión completa de la enfermedad al año del diagnóstico.


Lymphoma is the most common childhood malignancy in the head and neck. The most common clinical manifestations of lymphoma in palatine tonsil are the tonsils asymmetry, alteration in the appearance of the mucous and cervical lymphadenopathy. The unilateral tonsillar hypertrophy must be differentiated with infectious processes, with chronic processes, other tumors or benign tonsillar hyperplasia. The monitoring of tonsillar hypertrophy is strict, because early diagnosis and treatment are of great importance in the prognosis of tonsillar lymphoma patients. A 14 year-old girl presents unilateral tonsillar enlargement and odynophagia. After 21 days of antibiotic treatment, she has not clinical improvement. The diagnosis is Burkitt's lymphoma. One year after diagnosis, she presents complete remission of the disease.


Assuntos
Humanos , Feminino , Adolescente , Neoplasias Tonsilares , Linfoma de Burkitt/diagnóstico , Linfoma de Burkitt/terapia
6.
Blood Adv ; 5(14): 2852-2862, 2021 07 27.
Artigo em Inglês | MEDLINE | ID: mdl-34283175

RESUMO

Data addressing prognostication in patients with HIV related Burkitt lymphoma (HIV-BL) currently treated remain scarce. We present an international analysis of 249 (United States: 140; United Kingdom: 109) patients with HIV-BL treated from 2008 to 2019 aiming to identify prognostic factors and outcomes. With a median follow up of 4.5 years, the 3-year progression-free survival (PFS) and overall survival (OS) were 61% (95% confidence interval [CI] 55% to 67%) and 66% (95%CI 59% to 71%), respectively, with similar results in both countries. Patients with baseline central nervous system (CNS) involvement had shorter 3-year PFS (36%) compared to patients without CNS involvement (69%; P < .001) independent of frontline treatment. The incidence of CNS recurrence at 3 years across all treatments was 11% with a higher incidence observed after dose-adjusted infusional etoposide, doxorubicin, vincristine, prednisone, cyclophosphamide (DA-EPOCH) (subdistribution hazard ratio: 2.52; P = .03 vs other regimens) without difference by CD4 count 100/mm3. In multivariate models, factors independently associated with inferior PFS were Eastern Cooperative Oncology Group (ECOG) performance status 2-4 (hazard ratio [HR] 1.87; P = .007), baseline CNS involvement (HR 1.70; P = .023), lactate dehydrogenase >5 upper limit of normal (HR 2.09; P < .001); and >1 extranodal sites (HR 1.58; P = .043). The same variables were significant in multivariate models for OS. Adjusting for these prognostic factors, treatment with cyclophosphamide, vincristine, doxorubicin, and high-dose methotrexate, ifosfamide, etoposide, and high-dose cytarabine (CODOX-M/IVAC) was associated with longer PFS (adjusted HR [aHR] 0.45; P = .005) and OS (aHR 0.44; P = .007). Remarkably, HIV features no longer influence prognosis in contemporaneously treated HIV-BL.


Assuntos
Linfoma de Burkitt , Infecções por HIV , Linfoma de Burkitt/diagnóstico , Linfoma de Burkitt/tratamento farmacológico , Linfoma de Burkitt/epidemiologia , Intervalo Livre de Doença , Infecções por HIV/tratamento farmacológico , Infecções por HIV/epidemiologia , Humanos , Recidiva Local de Neoplasia , Rituximab , Reino Unido , Estados Unidos/epidemiologia
7.
Front Immunol ; 12: 647733, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34295326

RESUMO

There is limited experience of PD-1 antibody combined with other therapies in children. We aimed to explore the antitumor activity and safety of PD-1 antibody monotherapy or combination with other regimens in relapsed or refractory pediatric cancer. This is a retrospective-case study conducted in two Chinese expert centers. The primary objective of this study was to describe the overall response rate (ORR) and disease control rate (DCR). Secondary objectives included characterizing toxicities. Of the 22 pediatric patients with cancer who received PD-1 inhibitors, the median follow-up for all patients after the commencement of PD-1 therapy with or without other regimens was 12.3 months (0 - 43 months). PD-1 antibody monotherapy demonstrated antitumor activity in a population of pediatric patients with Hodgkin lymphoma (HL), with an objective response rate (ORR) and disease control rate (DCR) of 83.3% (3CR and 2PR) and 100%, respectively. However, no objective response was observed in patients with melanoma or Burkitt lymphoma evaluated in this study. We reviewed responses for patients with chemotherapy, decitabine or everolimus combination therapies with PD-1 antibodies, and found that PD-1 antibody combined with decitabine showed potential efficacy in pediatric patients with advanced embryonal rhabdomyosarcoma and lymphoepitheliomatoid-like carcinoma. There were no severe treatment-related adverse events (TRAEs) directly attributed to PD-1 antibody monotherapy in Asian pediatric patients with lower incidence of hematologic toxicity and nonhematologic toxicity. The Grade ≥3 TRAEs were attributed to the combination chemotherapy.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Antineoplásicos Imunológicos/uso terapêutico , Linfoma de Burkitt/terapia , Doença de Hodgkin/terapia , Inibidores de Checkpoint Imunológico/uso terapêutico , Imunoterapia/métodos , Melanoma/terapia , Receptor de Morte Celular Programada 1/antagonistas & inibidores , Neoplasias Cutâneas/terapia , Adolescente , Linfoma de Burkitt/epidemiologia , Linfoma de Burkitt/imunologia , Criança , Pré-Escolar , China/epidemiologia , Quimioterapia Combinada/métodos , Feminino , Seguimentos , Doença de Hodgkin/epidemiologia , Doença de Hodgkin/imunologia , Humanos , Lactente , Masculino , Melanoma/epidemiologia , Melanoma/imunologia , Estudos Retrospectivos , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/imunologia , Resultado do Tratamento
8.
Int J Mol Sci ; 22(14)2021 Jul 09.
Artigo em Inglês | MEDLINE | ID: mdl-34298992

RESUMO

Non-Hodgkin B-cell lymphomas (NHL) are a heterogeneous group of lymphoid neoplasms with complex etiopathology, rich symptomatology, and a variety of clinical courses, therefore requiring different therapeutic approaches. The hypothesis that an infectious agent may initiate chronic inflammation and facilitate B lymphocyte transformation and lymphogenesis has been raised in recent years. Viruses, like EBV, HTLV-1, HIV, HCV and parasites, like Plasmodium falciparum, have been linked to the development of lymphomas. The association of chronic Helicobacter pylori (H. pylori) infection with mucosa-associated lymphoid tissue (MALT) lymphoma, Borrelia burgdorferi with cutaneous MALT lymphoma and Chlamydophila psittaci with ocular adnexal MALT lymphoma is well documented. Recent studies have indicated that other infectious agents may also be relevant in B-cell lymphogenesis such as Coxiella burnettii, Campylobacter jejuni, Achromobacter xylosoxidans, and Escherichia coli. The aim of the present review is to provide a summary of the current literature on infectious bacterial agents associated with B-cell NHL and to discuss its role in lymphogenesis, taking into account the interaction between infectious agents, host factors, and the tumor environment.


Assuntos
Infecções Bacterianas/complicações , Linfoma de Burkitt/microbiologia , Infecções por Helicobacter/microbiologia , Interações Hospedeiro-Patógeno , Linfoma de Zona Marginal Tipo Células B/microbiologia , Linfoma Difuso de Grandes Células B/microbiologia , Infecções Bacterianas/imunologia , Linfoma de Burkitt/complicações , Linfoma de Burkitt/patologia , Carcinogênese/genética , Carcinogênese/imunologia , Carcinogênese/metabolismo , Infecções por Helicobacter/complicações , Helicobacter pylori/patogenicidade , Interações Hospedeiro-Patógeno/genética , Interações Hospedeiro-Patógeno/imunologia , Humanos , Linfoma de Zona Marginal Tipo Células B/complicações , Linfoma de Zona Marginal Tipo Células B/patologia , Linfoma Difuso de Grandes Células B/complicações , Linfoma Difuso de Grandes Células B/patologia , Microambiente Tumoral/genética , Microambiente Tumoral/imunologia
9.
Orphanet J Rare Dis ; 16(1): 331, 2021 07 30.
Artigo em Inglês | MEDLINE | ID: mdl-34330316

RESUMO

BACKGROUND: Mature B cell acute lymphoblastic leukaemia (BAL) is characterised by French-American-British (FAB)-L3 morphology and the presence of surface immunoglobulin (sIgM) light chain restriction. BAL is also considered as the leukaemic phase of Burkitt lymphoma (BL), in which t (8; 14) (q24; q32) or its variants are related to the myelocytomatosis oncogene (MYC) rearrangement (MYCr) is usually present. However, BAL with lysine methyltransferase 2A (KMT2A, previously called Mixed lineage leukaemia, MLL) gene rearrangement (KMT2Ar, previously called MLLr) is rare. RESULTS: Three BAL patients with KMT2Ar were enrolled between January 2017 and November 2019, accounting for 1.37% of the B-ALL population in our hospital. We also reviewed 24 previously reported cases of BAL and KMT2Ar and analysed the features, treatment, and prognosis. Total 13 males and 14 females were enrolled in our research, and the average age at diagnosis was 19.5 ± 4.95 months old. In these 27 patients, renal, central nervous system (CNS) and skin involvement were existent in 6, 4 and 3 patients, respectively; 26 patients (26/27) showed non-ALL-L3 morphology, while one patient is ALL-L3; overexpression of CD19 was detected in most cases, negative or suspicious expression of CD20 was found in 64% of patients. KMT2Ar was reported, but MYCr was not observed. 25 patients (25/27) achieved complete remission after chemotherapy or Stem cell transplantation. The patients were sensitive to chemotherapy, prospective event-free survival (pEFS) of BAL patients with KMT2Ar who received allogeneic haematopoietic stem cell transplantation (allo-HSCT) was higher than that in patients who received chemotherapy alone (83.33% vs 41.91%). CONCLUSION: BAL patients with KMT2Ar had unique manifestations, including younger age at diagnosis and overexpression of CD19; expression of CD20 was rare, and MYCr was undetectable. The pEFS was higher in patients undergoing allo-HSCT than in patients undergoing chemotherapy alone.


Assuntos
Linfoma de Burkitt , Transplante de Células-Tronco Hematopoéticas , Leucemia-Linfoma Linfoblástico de Células Precursoras , Linfócitos B , Criança , Pré-Escolar , Feminino , Rearranjo Gênico/genética , Humanos , Lactente , Masculino , Proteínas Nucleares , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Estudos Prospectivos
11.
Zhonghua Bing Li Xue Za Zhi ; 50(6): 604-608, 2021 Jun 08.
Artigo em Chinês | MEDLINE | ID: mdl-34078047

RESUMO

Objective: To investigate the clinicopathological features, molecular genetics, treatment and prognosis of Burkitt-like lymphoma with 11q aberration (BLL-11q). Methods: Six cases of BLL-11q diagnosed at the First Affiliated Hospital of Zhengzhou University, from January 2016 to January 2020 were reviewed and analyzed using hematoxylin-eosin staining, immunohistochemistry, EBER in situ hybridization and fluorescence in situ hybridization. Clinical information including follow-up data was collected and analyzed. Results: The median age of the six immunocompetent patients was 29 years (range 20-38 years) and the male to female ratio was 5∶1. All patients had nodal disease in the head and neck region. Five patients had Ann Arbor stage Ⅰ-Ⅱ disease, while one patient had stage Ⅳ disease. Lymph nodes showed partial or total architectural effacement by a diffuse proliferation of monomorphic lymphocytes. Four cases were morphologically similar to Burkitt lymphoma, and two cases were unclassified with histological features between Burkitt lymphoma and diffuse large B-cell lymphoma. Mitotic figures, apoptosis and necrosis were conspicuous. Five cases exhibited the"starry sky"pattern. CD20, CD10 and bcl-6 were diffusely and strongly positive. The Ki-67 index was more than 95%. The follicular-dendritic-cell meshwork was noted in one case using CD21 stain. C-MYC was expressed variably. CD3, bcl-2, MUM-1, CD30 and TDT were negative in all cases. EBER in situ hybridization was also all negative. FISH analyses using C-MYC, bcl-2 and bcl-6 break-apart probes were all negative. All cases had the 11q23.3 gain/11q24.3 loss pattern, and 11q23.3 amplification was found in one case. IgH and IRF4 break-apart probes analysis was also negative. All patients were alive with no disease after a follow-up of 4 to 19 months. Conclusion: BLL-11q is a rare lymphoma that resembles Burkitt lymphoma morphologically and phenotypically, but lacks C-MYC gene rearrangements. Instead, it has a chromosome-11q alteration characterized by proximal gains and telomeric losses. It's necessary to improve our understanding of BLL-11q to avoid misdiagnosis and missed diagnosis.


Assuntos
Linfoma de Burkitt , Linfoma Difuso de Grandes Células B , Adulto , Linfoma de Burkitt/genética , Aberrações Cromossômicas , Feminino , Genes myc/genética , Humanos , Hibridização in Situ Fluorescente , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Linfoma Difuso de Grandes Células B/genética , Masculino , Biologia Molecular , Translocação Genética , Adulto Jovem
13.
Artigo em Inglês | MEDLINE | ID: mdl-34070881

RESUMO

Background: Malaria infection is reportedly linked to endemic Burkitt lymphoma (eBL) in malaria-endemic areas. This study aimed to pool the overall risk (or odds) of eBL among children with previous or concurrent malaria infection. Methods: We searched PubMed, Web of Science, Scopus, and reference lists of publications for potentially relevant studies on malaria infection and eBL. The quality of the included studies was assessed using the Joanna Briggs Institute for case-control studies. Random-effects meta-analysis was used to summarize whether the odds of eBL can be increased by (1) malaria infection or (2) elevated titer of IgGs to malaria antigen. The level of heterogeneity was evaluated using Cochran's Q statistic and I2. The individual study data, pooled odds, and confidence interval (CI) were illustrated using the forest plot. Publication bias was assessed using funnel plots and Egger's test. Results: Ten studies were included, reporting the number of malaria cases in eBL and non-eBL (5 studied malaria infection and the odds of eBL; five studied the burden of IgGs to malarial antigens and the odds of eBL). According to the meta-analysis results, the odds of eBL was not increased by malaria infection (p = 0.562, OR: 0.87, 95% CI: 0.54-1.39, I2: 93.5%, malaria in eBL: 604/1506 cases, malaria in non-eBL: 2117/4549 cases) and the elevated titer of IgGs to malaria antigen (p = 0.051, OR: 1.50, 95% CI: 1.00-2.25, I2: 89%, increased IgG titer in eBL: 1059/1736 cases, increased IgG titer in non-eBL: 847/1722 cases). In meta-regression analysis, sex was not a confounding factor for the effect size of malaria infection and eBL (p = 0.10) and that of increased IgGs and eBL (p = 0.80). Conclusions: Malaria infection and IgG titer elevation did not increase the risk for eBL among children. However, the included studies, which are only few, do not generally agree on this point. Therefore, the risk for eBL in children diagnosed with malaria should be investigated further by longitudinal studies to confirm our evidence-based approach.


Assuntos
Linfoma de Burkitt , Malária , Linfoma de Burkitt/epidemiologia , Estudos de Casos e Controles , Criança , Humanos , Malária/epidemiologia
14.
J Oral Pathol Med ; 50(6): 572-586, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-34091974

RESUMO

BACKGROUND: Burkitt lymphoma (BL) is an aggressive B-cell lymphoma with three variants (endemic, sporadic, and immunodeficiency-associated), presenting with specific epidemiological and clinical features. Burkitt lymphoma affects the head and neck region (BLHN) in approximately 10% of cases. The aim of this study was to undertake a comparative analysis of the clinicopathologic and immunohistochemical (IHC) features of BLHN diagnosed in patients from Africa, Guatemala, and Brazil. METHODS: Cases diagnosed as BLHN were collected from the files of six oral pathology laboratory services (Brazil, South Africa, and Guatemala) and one Brazilian pediatric oncology hospital from 1986 to 2020. Clinicopathological and IHC data, and Epstein-Barr virus (EBV) status by in situ hybridization data for each case were reviewed and described. RESULTS: Of the 52 cases, BLHN was predominant in pediatric patients [43 (82.69%)] and males [43 (82.69%)], with a mean age of 11.26 ± 9.68 years (range, 1-39 years). Neck and cervical lymph nodes [14 (26.92%)], and involvement of both maxilla and mandible [8 (15.38%)], were the most common anatomical sites. Clinically, tumor/swelling [40 (31.25%)], cervical lymphadenopathy [14 (10.94%)], pain [12 (9.38%)], and bone destruction [12 (9.38%)] were frequent findings. All cases showed typical morphological characteristics of BL. IHC profiles included positivity for CD20 [52 (100%)], CD10 [38 (79.17%)], Bcl6 [29 (87.88%)], and c-Myc protein [18 (81.82%)]. EBV was positive in 18 cases (62.07%). The Ki-67 index ranged from 90 to 100%. CONCLUSION: The clinicopathological and EBV profile of BLHN in South African, Guatemalan, and Brazilian patients is similar.


Assuntos
Linfoma de Burkitt , Infecções por Vírus Epstein-Barr , Adolescente , Adulto , Brasil/epidemiologia , Linfoma de Burkitt/diagnóstico , Linfoma de Burkitt/epidemiologia , Criança , Pré-Escolar , Herpesvirus Humano 4 , Humanos , Lactente , Masculino , África do Sul/epidemiologia , Adulto Jovem
15.
J Clin Exp Hematop ; 61(2): 114-119, 2021 Jun 05.
Artigo em Inglês | MEDLINE | ID: mdl-33994432

RESUMO

A 62-year-old woman, who had a 16-year history of JAK2V617F-mutated myeloproliferative neoplasm (MPN), developed Burkitt leukemia (BL) 16 months after treatment with ruxolitinib to control hydroxyurea-refractory conditions. BL cells were CD10+, CD19+, CD20-, CD34-, cytoplasmic CD79a+, and TdT+, and lacked surface immunoglobulins but expressed the cytoplasmic µ heavy chain. In the bone marrow, nuclear MYC+ BL cells displaced the MPN tissues. t(8;14)(q24;q32) occurred at a CG dinucleotide within MYC exon 1 and at the IGHJ3 segment, and an N-like segment was inserted at the junction. The V-D-J sequence of the non-translocated IGH allele had the unmutated configuration. DNA from peripheral blood at a time of the course of MPN exhibited homozygous JAK2V617F mutation, while that at BL development included both JAK2V617F and wild-type DNAs. Although the association between JAK1/2 inhibitor therapy for MPN and secondary development of aggressive B-cell neoplasm remains controversial, this report suggests that, in selected patients, close monitoring of clonal B-cells in the BM is required before and during treatment with JAK1/2 inhibitors.


Assuntos
Linfoma de Burkitt/etiologia , Hidroxiureia/uso terapêutico , Transtornos Mieloproliferativos/tratamento farmacológico , Células Precursoras de Linfócitos B/patologia , Pirazóis/uso terapêutico , Linfoma de Burkitt/genética , Linfoma de Burkitt/patologia , Feminino , Humanos , Janus Quinase 2/genética , Pessoa de Meia-Idade , Transtornos Mieloproliferativos/genética , Transtornos Mieloproliferativos/patologia , Mutação Puntual
16.
Ann Ital Chir ; 92: 268-276, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34031279

RESUMO

INTRODUCTION: Intussusception is a common condition in children, it is rare in adults. Adult intussusception differs from pediatric intussusception in various respects, including etiology clinical characteristics and therapy. METHODS: We present and discuss a new case of intussusception in children and adults. RESULTS: In child the Barium Enema x-ray examination is identified an endoluminal filling defect to refer to the apex of the invaginated loop at the rectal level, with slow ascent during the progressive injection of the radiopaque contrast medium. At the end of the procedure, incomplete reduction of the picture is documented. The patient undergoes emergency surgery where the presence of an ileo-ceco-colic invagination is documented. Intussusception is reduced by taxis. In the adult laparoscopic right hemicolectomy was performed. High-grade B-cell Burkitt's lymphoma was confirmed by immunohistochemistry. DISCUSSION: In contrast to intussusceptions in children, in the adult population, a demonstrable etiology is found in most of the cases. In adults surgery is always indicated. The non-invasive resolutive intervention most commonly used in the child and best known consists in the rectal introduction of a radiopaque contrast medium (air or barium) at controlled pressure until. CONCLUSIONS: Although intussusceptions occur at all ages, there are major differences in the clinical presentation, diagnostic approach, and management between pediatric and adult populations. Intussusception is remarkably different in these two age groups and it must be approached from a different clinical perspective. KEY WORDS: Intussusception in children, Intussusception in adults, Intussusception symptoms, Radiology and treatment.


Assuntos
Linfoma de Burkitt , Doenças do Ceco , Doenças do Íleo , Intussuscepção , Adulto , Fatores Etários , Linfoma de Burkitt/complicações , Linfoma de Burkitt/diagnóstico por imagem , Linfoma de Burkitt/cirurgia , Doenças do Ceco/complicações , Doenças do Ceco/diagnóstico por imagem , Doenças do Ceco/cirurgia , Pré-Escolar , Colectomia , Humanos , Doenças do Íleo/complicações , Doenças do Íleo/diagnóstico por imagem , Doenças do Íleo/cirurgia , Valva Ileocecal/diagnóstico por imagem , Valva Ileocecal/cirurgia , Intussuscepção/diagnóstico por imagem , Intussuscepção/etiologia , Intussuscepção/cirurgia , Masculino
19.
BMJ Case Rep ; 14(4)2021 Apr 20.
Artigo em Inglês | MEDLINE | ID: mdl-33879463

RESUMO

Burkitt lymphoma (BL) is a rare, mature, fast-growing and highly aggressive B-cell neoplasm. It has a high-proliferation rate with distinctive genetic changes in the C-MYC oncogene. Treatment usually requires intense and frequent chemotherapy regimens with central nervous system prophylaxis as the usual regimens used for other non-Hodgkin's lymphoma yield poor survival in BL. This report discusses a patient who was diagnosed with a stage II, high-grade BL who received one cycle of intense chemotherapy and refused further treatment. That patient remained in complete remission in his last follow-up; 7 years from diagnosis without requiring other therapies for his lymphoma.


Assuntos
Linfoma de Burkitt , Linfoma de Células B , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Linfoma de Burkitt/diagnóstico , Linfoma de Burkitt/tratamento farmacológico , Humanos , Imunoterapia , Linfoma de Células B/tratamento farmacológico , Indução de Remissão
20.
Yakugaku Zasshi ; 141(4): 481-488, 2021.
Artigo em Japonês | MEDLINE | ID: mdl-33790114

RESUMO

Two novel ß-trefoil lectins, MytiLec-1 and SeviL were found from mussels in the coast of Yokohama and Nagasaki. MytiLec-1 was purified from gill and mantle of Mytilus galloprovincialis. It was consisted of 149 amino acid residues and there was no similarity with any other proteins when it was discovered. We advocate for this "Mytilectin" as a new protein family because of their novelty of its primary structure and homologues were also found in other mussels. Glycan array analysis revealed that MytiLec-1 specifically bound to the Gb3 and Gb4 glycan which contained the α-galactoside. MytiLec-1 caused the apoptosis against the Burkitt's lymphoma cells through the interaction of Gb3 express in their cell surface. On the other hand, SeviL obtained from gill and mantle of Mytilisepta virgata showed the specific binding against GM1b, asialo GM1 and SSEA-4 which are known as glycosphingolipid glycan including the ß-galactoside. In addition, SeviL was identified as R type lectin by confirmation of QXW motif within its primary structure. Messenger RNA of SeviL like R type lectins was also found among the musssels including Mytilus galloprovincialis. SeviL also showed the apoptosis against asialo GM1 expressing cells. To apply the anticancer lectin as a novel molecular target drug, primary structure of MytiLec-1 was analyzed to enhance the stabilization of confirmation by computational design technique. It was succeeded to produce a monomeric artificial ß-trefoil lectin, Mitsuba-1 without losing the Gb3 binding ability. Comparison of biological function between Mitsuba-1 and MytiLec-1 is also described in this study.


Assuntos
Dissacarídeos/farmacologia , Galectinas/farmacologia , Lectinas/farmacologia , Mytilidae/química , Trissacarídeos/farmacologia , Animais , Antineoplásicos , Linfoma de Burkitt/tratamento farmacológico , Linfoma de Burkitt/patologia , Dissacarídeos/química , Dissacarídeos/isolamento & purificação , Dissacarídeos/metabolismo , Desenho de Fármacos , Galectinas/química , Galectinas/isolamento & purificação , Galectinas/metabolismo , Lectinas/química , Lectinas/isolamento & purificação , Lectinas/metabolismo , Conformação Molecular , Terapia de Alvo Molecular , Polissacarídeos/metabolismo , Sequências de Repetição em Tandem , Trissacarídeos/química , Trissacarídeos/isolamento & purificação , Trissacarídeos/metabolismo
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