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[PMID]:29429164
[Au] Autor:Zhang F; Luo DL; Chen Y; Wu HM; Yan JH; Luo XL; He J; Luo LQ; Liu YH
[Ad] Endereço:Department of Pathology, Guangdong General Hospital/Guangdong Academy of Medical Sciences, Guangzhou 510080, China.
[Ti] Título:[Expression of ßF1 and T cell receptor γ in T lymphoblastic lymphoma/leukemia].
[So] Source:Zhonghua Bing Li Xue Za Zhi;47(2):119-122, 2018 Feb 08.
[Is] ISSN:0529-5807
[Cp] País de publicação:China
[La] Idioma:chi
[Ab] Resumo:To evaluate the expression of ßF1 and T cell receptor (TCR)γ in T lymphoblastic lymphoma/leukemia(T-LBL/ALL), and investigate the clinicopathological features. Fifty-one cases of T-LBL/ALL were collected at Guangdong General Hospital from 2010 to 2016, the expression of ßF1 and TCRγ was assessed by immunohistochemistry. There were 13 cases of children and adolescents, and 38 cases of adults. The expression rates of ßF1 and TCRγ were 27.5%(14/51) and 15.7%(8/51) respectively. The proportion of adults in αß T-LBL/ALL, TCR-silent T-LBL/ALL and γδ T-LBL/ALL was 7/14, 79.3%(23/29)and 8/8 respectively, and the difference was significant ( =0.023). There was no statistical difference in sex, LDH, bone marrow involvement and Ann arbor stage among these three groups( >0.05). γδ T-LBL/ALLs included 6 cases of CD4(-)/CD8(-) phenotype, whereas αß T-LBL/ALL included 7 cases of CD4(+) /CD8(+) phenotype. There was significant difference in CD4/CD8 expression among these three groups( <0.01). γδ T-LBL/ALL occurred only in adults, with predominantly CD4(-)/CD8(-) phenotype. αß T-LBL/ALL occurred more common in children and adolescents, with predominantly CD4(+) /CD8(+) phenotype.
[Mh] Termos MeSH primário: Linfoma de Células T/metabolismo
Leucemia-Linfoma Linfoblástico de Células T Precursoras/metabolismo
Receptores de Antígenos de Linfócitos T/metabolismo
[Mh] Termos MeSH secundário: Adolescente
Adulto
Criança
Seres Humanos
Imuno-Histoquímica
Fenótipo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Receptors, Antigen, T-Cell)
[Em] Mês de entrada:1803
[Cu] Atualização por classe:180306
[Lr] Data última revisão:
180306
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:180213
[St] Status:MEDLINE
[do] DOI:10.3760/cma.j.issn.0529-5807.2018.02.008


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[PMID]:29397596
[Au] Autor:Wang YN; Li J; Ni YH; Liu Y; Li Y; Zhang Y; Zhou WX; Fei GJ; Qian JM; Li JN
[Ti] Título:[The clinical characteristics of patients with monomorphic epitheliotropic intestinal T-cell lymphoma characterized by minor endoscopic abnormalities].
[So] Source:Zhonghua Nei Ke Za Zhi;57(2):112-117, 2018 Feb 01.
[Is] ISSN:0578-1426
[Cp] País de publicação:China
[La] Idioma:chi
[Ab] Resumo:To clarify the clinical features of monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) with minor endoscopic abnormalities. The clinical data of 6 patients with MEITL characterized by minor endoscopic abnormalities in Peking Union Medical College Hospital from 2012 to 2016 were retrospectively analyzed, including clinical manifestations, endoscopic, pathological features, medications and prognosis. Five out of 6 patients were male, with an average age of 61.2 years old. The median disease duration was 4.5 months. All patients initially presented with diarrhea without specific findings for serologic testing. CT enterography showed continuous intestinal lesions, including symmetric thickening of the bowel wall, abnormal hyperenhancement of mucosal surface and lymphadenopathy. Endoscopic appearances were only mildly abnormal, including mucosal swelling, atrophy of villus, mosaic sign and shallow ulcers. Histopathologic findings revealed massive small to medium sized T lymphocytes infiltration with positive expression of CD(3) and CD(8). Chemotherapy and palliative treatment were administrated after diagnosis. Clinical presentations of MEITL are non-specific with minor endoscopic abnormalities. Therefore, biopsy is indispensable for patients with a relatively normal endoscopic result.
[Mh] Termos MeSH primário: Diarreia/etiologia
Endoscopia
Intestinos/diagnóstico por imagem
Linfadenopatia/diagnóstico por imagem
Linfoma de Células T/diagnóstico
[Mh] Termos MeSH secundário: Idoso
Antineoplásicos/uso terapêutico
Biópsia
Feminino
Seres Humanos
Linfoma de Células T/tratamento farmacológico
Masculino
Meia-Idade
Estudos Retrospectivos
Linfócitos T
Tomografia Computadorizada por Raios X
Resultado do Tratamento
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Antineoplastic Agents)
[Em] Mês de entrada:1803
[Cu] Atualização por classe:180306
[Lr] Data última revisão:
180306
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:180206
[St] Status:MEDLINE
[do] DOI:10.3760/cma.j.issn.0578-1426.2018.02.006


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[PMID]:29395854
[Au] Autor:Sarradin V; Simon L; Huynh A; Gilhodes J; Filleron T; Izar F
[Ad] Endereço:Department of radiotherapy, IUCT-Oncopole, 1, avenue Irène-Joliot-Curie, 31000 Toulouse, France.
[Ti] Título:Total body irradiation using Helical Tomotherapy : Treatment technique, dosimetric results and initial clinical experience.
[So] Source:Cancer Radiother;22(1):17-24, 2018 Feb.
[Is] ISSN:1769-6658
[Cp] País de publicação:France
[La] Idioma:eng
[Ab] Resumo:PURPOSE: Helical TomoTherapy allows precise and homogeneous tumour coverage and excellent sparing of organs at risk. We present here our treatment technique, dosimetric results, and our first clinical data for patients receiving total body irradiation as part of the conditioning regimen before hematopoietic stem cell transplantation. PATIENTS AND METHODS: The cohort consisted of 11 patients who were treated in our institution between August 2014 and January 2016. The total dose was 12Gy in six fractions in three days. We collected the dose distribution information in the treatment volumes, organs at risk and area of junction. We report retrospectively the clinical events during the first 6 months after the procedure. RESULTS: Median age was 31 years (range, 18-57 years). Median D98% was 11.5Gy (range: 6.6-11.9Gy). The median of the mean doses to the lungs was 8.7Gy (range: 8.5-9.3Gy). The mean dose for the junction area was 12Gy (range: 11.9-12.1Gy). All patients had the total procedure, and all underwent successful engraftment. During the first six months, nine patients had at least one grade 3 or 4 toxicity that was due essentially to graft versus host disease. No patient had radiation pneumonitis. The toxicities were both more frequent and of higher grade during the first three months. CONCLUSION: Total body irradiation using helical TomoTherapy is feasible. It allows a very good homogeneity of dose and conformity with an acceptable tolerance. It could deliver higher doses to sites at high risk of recurrence (bone marrow, sanctuary sites), while sparing major normal organs like lungs, liver, and kidneys. This reduction of dose could lead to reduced severity and frequency of late complications.
[Mh] Termos MeSH primário: Radioterapia de Intensidade Modulada
Irradiação Corporal Total
[Mh] Termos MeSH secundário: Adolescente
Adulto
Estudos de Coortes
Feminino
Seres Humanos
Leucemia/terapia
Linfoma de Células T/terapia
Masculino
Meia-Idade
Tratamentos com Preservação do Órgão
Órgãos em Risco
Dosagem Radioterapêutica
Radioterapia de Intensidade Modulada/métodos
Estudos Retrospectivos
Transplante de Células-Tronco
Irradiação Corporal Total/métodos
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180220
[Lr] Data última revisão:
180220
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:180204
[St] Status:MEDLINE


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[PMID]:28450574
[Au] Autor:Jaccard A; Hermine O
[Ad] Endereço:CENTRE HOSPITALIER UNIVERSITAIRE LIMOGES.
[Ti] Título:A major turning point in NK/T-cell lymphoma?
[So] Source:Blood;129(17):2342-2343, 2017 04 27.
[Is] ISSN:1528-0020
[Cp] País de publicação:United States
[La] Idioma:eng
[Mh] Termos MeSH primário: Linfoma de Células T Periférico
Linfoma de Células T
[Mh] Termos MeSH secundário: Seres Humanos
Células Matadoras Naturais
Neoplasias Nasais
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T; COMMENT
[Em] Mês de entrada:1801
[Cu] Atualização por classe:180116
[Lr] Data última revisão:
180116
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170429
[St] Status:MEDLINE
[do] DOI:10.1182/blood-2017-03-769075


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[PMID]:29310365
[Au] Autor:Sherkat R; Sabri MR; Dehghan B; Bigdelian H; Reisi N; Afsharmoghadam N; Rahimi H; Rahmanian N; Klein C
[Ad] Endereço:Acquired Immunodeficiency Research Center.
[Ti] Título:EBV lymphoproliferative-associated disease and primary cardiac T-cell lymphoma in a STK4 deficient patient: A case report.
[So] Source:Medicine (Baltimore);96(48):e8852, 2017 Dec.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:RATIONALE: Primary cardiac lymphoma (PLC) is an extremely uncommon malignancy. PCL is more common in secondary immunodeficient patients. In this report, we describe a unique case of PLC who had been diagnosed as a STK4 deficient patient. This case is the first Primary immunodeficiency (PID) patient developing PCL in the world. PATIENT CONCERNS: An eleven-year-old girl, a known case of PID, was referred to the pediatric cardiology department because of chest pain and dyspnea. Her CXR revealed cardiomegaly without mediastinal involvement and the echocardiography showed a mild pericardial effusion and cystic-shape echogenic masses. DIAGNOSES: After a period of missed follow up, she presented with respiratory distress following with syncope at the clinic because of a pressure effect of a large mass on the right ventricular outflow tract (RVOT) .An emergency operation was done for debulking of the tumors and resolving of RVOT obstruction. Biopsy and immunohistochemical staining was revealing "T-cell lymphoma", non-Hodgkin's type. INTERVENTIONS: Chemotherapy was done with cyclophosphamide, methotrexate, adriamycine, vincristine, hydrocortisone and allopurinol. OUTCOMES: The tumors shrank after chemotherapy initiation and she stayed stable for almost one month. Finally, she developed sever thrombocytopenia during her chemotherapy and died because of lung hemorrhage two months after her operation. LESSONS: Although PCL is very rare, it must be considered in the differential diagnosis of intracardiac mass or refractory pericardial effusions, especially in PIDs which are widely known for developing EBV-associated diseases such as lymphoma.
[Mh] Termos MeSH primário: Neoplasias Cardíacas/imunologia
Neoplasias Cardíacas/terapia
Linfoma de Células T/imunologia
Linfoma de Células T/terapia
Transtornos Linfoproliferativos/imunologia
Proteínas Serina-Treonina Quinases/deficiência
[Mh] Termos MeSH secundário: Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico
Criança
Terapia Combinada
Ecocardiografia
Evolução Fatal
Feminino
Seres Humanos
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
EC 2.7.1.- (STK4 protein, human); EC 2.7.11.1 (Protein-Serine-Threonine Kinases)
[Em] Mês de entrada:1801
[Cu] Atualização por classe:180115
[Lr] Data última revisão:
180115
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:180110
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000008852


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[PMID]:28744576
[Au] Autor:Wang Y; Li Q; Zhu L; Mao X; Zhang H; Huang L; Meng F; Wei J
[Ad] Endereço:Department of Hematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China.
[Ti] Título:Cytogenetics with flow cytometry in lymph node/extranodal tissue biopsies is sensitive to assist the early diagnosis of suspected lymphomas.
[So] Source:Ann Hematol;96(10):1673-1680, 2017 Oct.
[Is] ISSN:1432-0584
[Cp] País de publicação:Germany
[La] Idioma:eng
[Ab] Resumo:Few studies have examined the value of cytogenetic studies with flow cytometry (FC) in lymph node/extranodal tissue biopsies with suspected lymphoma. To evaluate this, G-banded karyotyping and/or fluorescence in situ hybridization (FISH) with FC immunophenotyping were performed on 185 lymph node or extranodal tissue biopsy specimens with suspected lymphoma. Complete cytogenetic analysis of lymph node/extranodal tissue was successful in 174 cases (94.1%) and 57.5% demonstrated chromosomal abnormalities. In 116 malignant lymphoma cases, 83.8% showed abnormalities. In 74 B cell lymphomas (B-NHL), abnormalities were more frequent in lymph node/extranodal tissues than in bone marrow by conventional cytogenetics (CC, 97.2 vs 26.1%), FISH (70.6 vs 17.6%), and FC (98.6 vs 28.4%). Three B-NHL diagnoses were confirmed by re-biopsy of lymph nodes due to the presence of abnormalities in the first biopsy, but no evidence of malignancy in pathological, FC, or IgH/TCR gene rearrangement analyses. In 29 T cell lymphomas (T-NHL), abnormalities were more frequent in lymph nodes than in bone marrow by CC (67.9 vs 21.4%) and FC (75.9 vs 27.6%) analyses. As expected, in 13 Hodgkin lymphoma cases, abnormalities were more frequent in lymph nodes than bone marrow by CC (41.7 vs 16.7%) and FC (30.8 vs 7.7%) analyses. In 56 reactive lymphoid hyperplasias (RLH), 7.1% had conventional clonal cytogenetic abnormalities. Two of these patients died of disease progression and two had their pathological diagnosis revised after the second review. These findings indicate that cytogenetic analysis combined with FC in lymph node/extranodal tissue biopsies can provide critical information in the auxiliary diagnosis of lymphoma.
[Mh] Termos MeSH primário: Aberrações Cromossômicas
Citometria de Fluxo
Rearranjo Gênico
Doença de Hodgkin
Linfoma de Células B
Linfoma de Células T
[Mh] Termos MeSH secundário: Adolescente
Adulto
Idoso
Idoso de 80 Anos ou mais
Feminino
Doença de Hodgkin/diagnóstico
Doença de Hodgkin/genética
Doença de Hodgkin/patologia
Seres Humanos
Linfoma de Células B/diagnóstico
Linfoma de Células B/genética
Linfoma de Células B/patologia
Linfoma de Células T/diagnóstico
Linfoma de Células T/genética
Linfoma de Células T/patologia
Masculino
Meia-Idade
Biópsia de Linfonodo Sentinela
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171129
[Lr] Data última revisão:
171129
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170727
[St] Status:MEDLINE
[do] DOI:10.1007/s00277-017-3066-y


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[PMID]:29072952
[Au] Autor:Hathuc VM; Hristov AC; Smith LB
[Ad] Endereço:From the Sections of Hematopathology (Drs Hathuc and Smith) and Dermatopathology (Dr Hristov) in the Department of Pathology, University of Michigan Medical Center, Ann Arbor.
[Ti] Título:Primary Cutaneous Acral CD8 T-Cell Lymphoma.
[So] Source:Arch Pathol Lab Med;141(11):1469-1475, 2017 Nov.
[Is] ISSN:1543-2165
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Primary cutaneous acral CD8 T-cell lymphoma is a new provisional entity in the 2016 revision of the World Health Organization classification of lymphoid neoplasms. This is a challenging diagnosis because of its rarity, as well as its morphologic and immunophenotypic overlap with other CD8 cytotoxic lymphoid proliferations. Appropriate classification of this entity is crucial because of its indolent clinical behavior compared with other CD8 T-cell lymphomas. Knowledge of the clinical setting, sites of involvement, and morphologic features can aid in correct diagnosis. Here, we review the clinical and pathologic features of primary cutaneous acral CD8 T-cell lymphoma with an emphasis on the differential diagnosis among other C8 T-cell lymphomas.
[Mh] Termos MeSH primário: Linfócitos T CD8-Positivos/patologia
Linfoma Cutâneo de Células T/diagnóstico
[Mh] Termos MeSH secundário: Biomarcadores Tumorais/metabolismo
Linfócitos T CD8-Positivos/metabolismo
Diagnóstico Diferencial
Extremidades
Seres Humanos
Imuno-Histoquímica/tendências
Imunofenotipagem/tendências
Linfoma de Células T/diagnóstico
Linfoma de Células T/metabolismo
Linfoma de Células T/patologia
Linfoma Cutâneo de Células T/metabolismo
Linfoma Cutâneo de Células T/patologia
Linfoma Cutâneo de Células T/terapia
Paniculite/diagnóstico
Paniculite/metabolismo
Paniculite/patologia
Prognóstico
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
0 (Biomarkers, Tumor)
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171106
[Lr] Data última revisão:
171106
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:171027
[St] Status:MEDLINE
[do] DOI:10.5858/arpa.2017-0230-RA


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[PMID]:28973700
[Au] Autor:Alsadi A; Lin D; Alnajar H; Brickman A; Martyn C; Gattuso P
[Ad] Endereço:From the Department of Pathology, Rush University Medical Center, Chicago, Illinois.
[Ti] Título:Hematologic Malignancies Discovered on Investigation of Breast Abnormalities.
[So] Source:South Med J;110(10):614-620, 2017 Oct.
[Is] ISSN:1541-8243
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:OBJECTIVES: Hematological malignancies of the breast share a presentation similar to primary breast carcinomas but differ substantially in therapeutic approach and clinical outcomes. In this study, we investigate the frequency of hematological malignancies, their relative primary and secondary occurrences, and further characterize the distinct histopathologies of these malignancies with a special focus on lymphomas. To our knowledge this is one of the largest and most comprehensive studies of breast hematologic malignancies. METHODS: We conducted a retrospective review of our institution's pathology database for hematologic neoplasms diagnosed in breast tissue during a period of 22 years (1992-2014). Clinical characteristics, patient history, histologic subtype, and patient outcomes were analyzed. RESULTS: We identified 52 cases; 46 lymphomas, 4 plasmacytomas, and 2 myeloid sarcomas. The lymphoma cases were 15 diffuse large B-cell lymphomas (DLBCLs), 14 follicular lymphomas (FLs), 8 marginal zone lymphomas (MZLs), 2 anaplastic large T-cell lymphomas, 2 peripheral T-cell lymphomas-not otherwise specified, 1 each of small lymphocytic lymphoma, Burkitt lymphoma, mantle cell lymphoma, B-cell lymphoblastic lymphoma, and T-cell lymphoblastic lymphoma. In total, 30 cases were primary and 22 cases were secondary to the breast. Primary lymphomas accounted for 60% of lymphomas. Most FLs and almost all MZLs were primary. CONCLUSIONS: Primary hematological malignancies of the breast are more common than secondary: 58 % versus 42%. This finding is more evident in lymphomas: 63% versus 37%. The most common hematological malignancy in our study was DLBCL, followed by FL and MZL. Most FLs and almost all MZLs were primary. At the same time, the percentage of primary DLBCLs in our study is lower than the percentage reported in previous studies. We suggest that this could be the result of transformation from low-grade lymphomas. Although rare, hematological malignancies of the breast warrant a higher level of clinical suspicion as they present similarly to breast carcinomas but require a substantially different therapeutic approach.
[Mh] Termos MeSH primário: Neoplasias da Mama/diagnóstico
Carcinoma/diagnóstico
Neoplasias Hematológicas/diagnóstico
Linfoma/diagnóstico
Plasmocitoma/diagnóstico
Sarcoma Mieloide/diagnóstico
[Mh] Termos MeSH secundário: Adulto
Idoso
Idoso de 80 Anos ou mais
Animais
Neoplasias da Mama/patologia
Neoplasias da Mama/secundário
Linfoma de Burkitt/diagnóstico
Linfoma de Burkitt/patologia
Diagnóstico Diferencial
Feminino
Neoplasias Hematológicas/patologia
Seres Humanos
Leucemia Linfocítica Crônica de Células B/diagnóstico
Leucemia Linfocítica Crônica de Células B/patologia
Linfoma/patologia
Linfoma de Zona Marginal Tipo Células B/diagnóstico
Linfoma de Zona Marginal Tipo Células B/patologia
Linfoma Folicular/diagnóstico
Linfoma Folicular/patologia
Linfoma Difuso de Grandes Células B/diagnóstico
Linfoma Difuso de Grandes Células B/patologia
Linfoma Anaplásico de Células Grandes/diagnóstico
Linfoma Anaplásico de Células Grandes/patologia
Linfoma de Célula do Manto/diagnóstico
Linfoma de Célula do Manto/patologia
Linfoma de Células T/diagnóstico
Linfoma de Células T/patologia
Meia-Idade
Plasmocitoma/patologia
Leucemia-Linfoma Linfoblástico de Células Precursoras B/diagnóstico
Leucemia-Linfoma Linfoblástico de Células Precursoras B/patologia
Leucemia-Linfoma Linfoblástico de Células T Precursoras/diagnóstico
Leucemia-Linfoma Linfoblástico de Células T Precursoras/patologia
Estudos Retrospectivos
Sarcoma Mieloide/patologia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171010
[Lr] Data última revisão:
171010
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:171004
[St] Status:MEDLINE
[do] DOI:10.14423/SMJ.0000000000000710


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[PMID]:28899870
[Au] Autor:Loughran SJ; Comoglio F; Hamey FK; Giustacchini A; Errami Y; Earp E; Göttgens B; Jacobsen SEW; Mead AJ; Hendrich B; Green AR
[Ad] Endereço:Department of Haematology, Cambridge Institute for Medical Research, University of Cambridge, Cambridge, England, UK.
[Ti] Título:Mbd3/NuRD controls lymphoid cell fate and inhibits tumorigenesis by repressing a B cell transcriptional program.
[So] Source:J Exp Med;214(10):3085-3104, 2017 Oct 02.
[Is] ISSN:1540-9538
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Differentiation of lineage-committed cells from multipotent progenitors requires the establishment of accessible chromatin at lineage-specific transcriptional enhancers and promoters, which is mediated by pioneer transcription factors that recruit activating chromatin remodeling complexes. Here we show that the Mbd3/nucleosome remodeling and deacetylation (NuRD) chromatin remodeling complex opposes this transcriptional pioneering during B cell programming of multipotent lymphoid progenitors by restricting chromatin accessibility at B cell enhancers and promoters. Mbd3/NuRD-deficient lymphoid progenitors therefore prematurely activate a B cell transcriptional program and are biased toward overproduction of pro-B cells at the expense of T cell progenitors. The striking reduction in early thymic T cell progenitors results in compensatory hyperproliferation of immature thymocytes and development of T cell lymphoma. Our results reveal that Mbd3/NuRD can regulate multilineage differentiation by constraining the activation of dormant lineage-specific enhancers and promoters. In this way, Mbd3/NuRD protects the multipotency of lymphoid progenitors, preventing B cell-programming transcription factors from prematurely enacting lineage commitment. Mbd3/NuRD therefore controls the fate of lymphoid progenitors, ensuring appropriate production of lineage-committed progeny and suppressing tumor formation.
[Mh] Termos MeSH primário: Linfócitos B/metabolismo
Carcinogênese/metabolismo
Linhagem da Célula/fisiologia
Proteínas de Ligação a DNA/fisiologia
Linfócitos/fisiologia
Complexo Mi-2 de Remodelação de Nucleossomo e Desacetilase/fisiologia
Fatores de Transcrição/fisiologia
[Mh] Termos MeSH secundário: Animais
Diferenciação Celular/fisiologia
Regulação da Expressão Gênica/fisiologia
Linfoma de Células T/etiologia
Camundongos
Camundongos Endogâmicos C57BL
Células-Tronco Multipotentes/fisiologia
Timócitos/metabolismo
Timócitos/fisiologia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (DNA-Binding Proteins); 0 (Mbd3 protein, mouse); 0 (Transcription Factors); EC 3.5.1.98 (Mi-2 Nucleosome Remodeling and Deacetylase Complex)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171024
[Lr] Data última revisão:
171024
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170914
[St] Status:MEDLINE
[do] DOI:10.1084/jem.20161827


  10 / 6047 MEDLINE  
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[PMID]:28885361
[Au] Autor:Yamamoto M; Ikuta K; Toki Y; Hatayama M; Shindo M; Torimoto Y; Okumura T
[Ad] Endereço:aDivision of Gastroenterology and Hematology/Oncology, Department of Medicine bOncology Center, Asahikawa Medical University Hospital, Asahikawa, Japan.
[Ti] Título:Angioimmunoblastic T-cell lymphoma and hypereosinophilic syndrome with FIP1L1/PDGFRA fusion gene effectively treated with imatinib: A case report.
[So] Source:Medicine (Baltimore);96(36):e8001, 2017 Sep.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:RATIONALE: Hypereosinophilic syndrome (HES) is a rare disorder characterized by hypereosinophilia and organ damage. Some cases of HES are caused by the FIP1L1/PDGFRA fusion gene and respond to imatinib. FIP1L1/PDGFRA-positive HES occasionally evolves into chronic eosinophilic leukemia or into another form of myeloproliferative neoplasm; however, the development of a malignant lymphoma is very rare. We present a rare case of angioimmunoblastic T-cell lymphoma (AITL) and HES with the FIP1L1/PDGFRA gene rearrangement. PATIENT CONCERNS: A man in his 30s presented to our hospital with fever, hypereosinophilia, widespread lymphadenopathy, and splenomegaly. Laboratory tests showed hypereosinophilia, increased soluble interleukin-2 receptor, and increased vitamin B12. Positron-emission tomography with F fluorodeoxyglucose (FDG) showed positive FDG uptake in multiple enlarged lymph nodes throughout the body and the red bone marrow. A bone-marrow biopsy showed hypereosinophilia without dysplasia and an increased number of blasts. The FIP1L1/PDGFRA fusion gene was positive upon fluorescence in situ hybridization (FISH) analysis of the peripheral blood. Furthermore, biopsy of a lymph node from the neck revealed restiform hyperplasia of capillary vessels, with small lymphoma cells arranged around the capillaries. Lymphoma cells were positive for CD3, CD4, and CD10, and negative for CD20. Lymphoma cells were also positive for the FIP1L1/PDGFRA fusion gene by FISH analysis. DIAGNOSES: From these findings, the patient was diagnosed with HES and AITL with FIP1L1/PDGFRA. INTERVENTIONS: After the diagnosis, corticosteroid was administered but was ineffective. Imatinib was then administered. OUTCOMES: Imatinib was very effective for treating HES and AITL, and complete remission was achieved in both. LESSONS: This report presents the first case in which the FIP1L1/PDGFRA fusion gene was positive both in peripheral blood and lymph nodes, implying the possibility that the tumor cells acquired the FIP1L1/PDGFRA fusion gene in the early stage of hematopoietic progenitor cell developments. Imatinib was very effective in treating both HES and lymphoma, suggesting that the FIP1L1/PDGFRA fusion gene plays a key role in the pathogenesis of both HES and lymphoma.
[Mh] Termos MeSH primário: Antineoplásicos/uso terapêutico
Síndrome Hipereosinofílica/complicações
Mesilato de Imatinib/uso terapêutico
Linfoma de Células T/complicações
Proteínas de Fusão Oncogênicas/genética
[Mh] Termos MeSH secundário: Adulto
Diagnóstico Diferencial
Seres Humanos
Síndrome Hipereosinofílica/tratamento farmacológico
Síndrome Hipereosinofílica/genética
Síndrome Hipereosinofílica/patologia
Linfoma de Células T/tratamento farmacológico
Linfoma de Células T/genética
Linfoma de Células T/patologia
Masculino
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Antineoplastic Agents); 0 (FIP1L1-RARA fusion protein, human); 0 (Oncogene Proteins, Fusion); 8A1O1M485B (Imatinib Mesylate)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170918
[Lr] Data última revisão:
170918
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170909
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000008001



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