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[PMID]:28629605
[Au] Autor:Kiguchi T; Sato C; Takai K; Nakai Y; Kaneko Y; Matsuki M
[Ad] Endereço:Department of Diagnostic Radiology, Ichinomiya Nishi Hospital, 1-Hira Kaimei, Ichinomiya City, Aichi 494-0001, Japan; Department of Diagnostic Radiology, Niigata City General Hospital, 463-7 Shumoku, Chuo-ku, Niigata City, Niigata 950-1197, Japan. Electronic address: takakig+jo@gmail.com.
[Ti] Título:CT findings in 11 patients with TAFRO syndrome: a variant of multicentric Castleman's disease.
[So] Source:Clin Radiol;72(10):905.e1-905.e5, 2017 Oct.
[Is] ISSN:1365-229X
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:AIM: To assess detailed computed tomography (CT) findings in patients with the recently described thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly (TAFRO) syndrome, in order to contribute to imaging interpretation in the challenging diagnosis of this disease. MATERIALS AND METHODS: The institutional review board approved this retrospective study and waived the need for informed consent. Eleven patients (six men, five women; mean age, 52.5 years) with confirmed TAFRO syndrome were included in this study. Chest-to-pelvis CT images were analysed for the presence of anasarca, organomegaly, bone lesions, and lung lesions. RESULTS: Anasarca was present in all patients and involved multiple cavities and tissues; pleural effusion and ascites were found in 100% of patients; pericardial effusion in 64%; periportal collar in 91%; gallbladder wall oedema in 78%; subcutaneous oedema in 91%; retroperitoneal oedema in 100%; and mesenteric oedema in 100%. Organomegaly involved multiple organs: hepatomegaly in 73%, splenomegaly in 82%, lymphadenopathy in 100%, and enlarged anterior mediastinum in 64% (solitary, well-circumscribed mass, 0%; infiltrative mass, 0%; non-mass-forming infiltrative lesion, 64%). Bone lesions were present in 91% patients and all bone lesions had ground-glass density with diffuse distribution. None of the patients had any lesions in their lungs. CONCLUSION: The present study revealed that the findings of anasarca, organomegaly, and diffuse bony ground-glass appearance were observed in detail on CT in patients with TAFRO syndrome. A "matted" appearance of the enlarged anterior mediastinum is the characteristic CT finding of TAFRO syndrome, and it is possible to diagnose TAFRO syndrome from the combination of several CT findings.
[Mh] Termos MeSH primário: Doença de Castleman/diagnóstico por imagem
Edema/diagnóstico por imagem
Trombocitopenia/diagnóstico por imagem
Tomografia Computadorizada por Raios X
[Mh] Termos MeSH secundário: Adulto
Idoso
Doença de Castleman/patologia
Edema/complicações
Edema/patologia
Feminino
Febre/complicações
Febre/patologia
Fibrose/complicações
Fibrose/diagnóstico por imagem
Fibrose/patologia
Seres Humanos
Masculino
Meia-Idade
Reprodutibilidade dos Testes
Reticulina
Estudos Retrospectivos
Síndrome
Trombocitopenia/complicações
Trombocitopenia/patologia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Reticulin)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:171116
[Lr] Data última revisão:
171116
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170621
[St] Status:MEDLINE


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[PMID]:28502946
[Au] Autor:Nara M; Komatsuda A; Itoh F; Kaga H; Saitoh M; Togashi M; Kameoka Y; Wakui H; Takahashi N
[Ad] Endereço:Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan.
[Ti] Título:Two Cases of Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Failure, and Organomegaly (TAFRO) Syndrome with High Serum Procalcitonin Levels, Including the First Case Complicated with Adrenal Hemorrhaging.
[So] Source:Intern Med;56(10):1247-1252, 2017.
[Is] ISSN:1349-7235
[Cp] País de publicação:Japan
[La] Idioma:eng
[Ab] Resumo:Thrombocytopenia, Anasarca, Fever, Reticulin fibrosis/Renal failure, and Organomegaly (TAFRO) syndrome is a recently described systemic inflammatory disorder characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly. It has an acute or subacute onset of unknown etiology, although some pathological features resemble those of multicentric Castleman disease. We here report two cases of TAFRO syndrome. The symptoms and pathological findings in these cases met the 2015 diagnostic criteria. Our cases showed high serum procalcitonin levels, suggesting bacterial infection as an onset trigger. In addition, Case 1 is the first case complicated with adrenal hemorrhaging. Case 2 is the second case of tocilizumab-resistant TAFRO syndrome successfully treated with rituximab.
[Mh] Termos MeSH primário: Anticorpos Monoclonais Humanizados/uso terapêutico
Edema/diagnóstico
Febre/diagnóstico
Fibrose/diagnóstico
Síndrome POEMS/diagnóstico
Insuficiência Renal/diagnóstico
Trombocitopenia/diagnóstico
[Mh] Termos MeSH secundário: Doenças das Glândulas Suprarrenais/complicações
Adulto
Grupo com Ancestrais do Continente Asiático
Calcitonina/sangue
Edema/tratamento farmacológico
Feminino
Febre/tratamento farmacológico
Fibrose/tratamento farmacológico
Hemorragia/tratamento farmacológico
Hemorragia/etiologia
Seres Humanos
Masculino
Meia-Idade
Síndrome POEMS/tratamento farmacológico
Insuficiência Renal/tratamento farmacológico
Reticulina
Trombocitopenia/tratamento farmacológico
Trombocitopenia/terapia
Resultado do Tratamento
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Antibodies, Monoclonal, Humanized); 0 (Reticulin); 9007-12-9 (Calcitonin); I031V2H011 (tocilizumab)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171016
[Lr] Data última revisão:
171016
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170516
[St] Status:MEDLINE
[do] DOI:10.2169/internalmedicine.56.7991


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[PMID]:28435987
[Au] Autor:Eren R; Dogu MH; Emir S; Huq GE; Okçu O; Altindal S; Yokus O; Suyani E
[Ad] Endereço:Department of Hematology, Istanbul Training and Research Hospital, P.K.34098, Fatih, Istanbul, Turkey.
[Ti] Título:Rethinking the usefulness of bone marrow biopsy on treatment decision in CLL patients at diagnosis.
[So] Source:Ann Hematol;96(8):1315-1321, 2017 Aug.
[Is] ISSN:1432-0584
[Cp] País de publicação:Germany
[La] Idioma:eng
[Ab] Resumo:We aimed to investigate the role of bone marrow infiltration pattern (BMIP) and bone marrow reticulin fibrosis (BMRF) in determining treatment demand in patients with diagnosis of chronic lymphocytic leukemia (CLL). We retrospectively evaluated the data of 65 patients, who were followed with the diagnosis of CLL at Istanbul Training and Research Hospital, Department of Hematology, between July 2007 and June 2016. The median age of the patients was 64 years (range, 32-83). Twenty-three (35.4%) patients were female, and 42 (64.6%) were male. Early/mild grade BMRF was observed in 46 (70.8%) patients and advanced grade BMRF in 19 (29.2%) patients. Eleven (23.9%) of 46 patients with early/mild grade BMRF and 10 (52.9%) of 19 patients with advanced grade BMRF required treatment during follow-up (p = 0.04). According to the BMIP, 14 (21.5%) patients had diffuse and 51 (78.5%) patients had non-diffuse BMIP. Eleven (78.6%) of 14 patients with diffuse BMIP and 10 (19.6%) of 51 patients with non-diffuse BMIP required treatment during follow-up (p < 0.001). In univariate analysis, both advanced grade BMRF and diffuse BMIP had an impact on occurrence of treatment demand (p = 0.028, HR = 3.535 vs. p < 0.01 HR = 15.033). Multivariate analysis also revealed diffuse BMIP to be effective (p < 0.001, HR 13.089), while advanced grade BMRF failed to significantly influence treatment demand (p = 0.140, HR 2.664). In conclusion, in the light of our findings, it is reasonable to consider that bone marrow biopsy at the time of diagnosis might provide a preliminary information about treatment demand in patients with CLL.
[Mh] Termos MeSH primário: Doenças da Medula Óssea/patologia
Exame de Medula Óssea/métodos
Medula Óssea/patologia
Leucemia Linfocítica Crônica de Células B/terapia
[Mh] Termos MeSH secundário: Adulto
Idoso
Idoso de 80 Anos ou mais
Biópsia
Medula Óssea/metabolismo
Doenças da Medula Óssea/metabolismo
Doenças da Medula Óssea/terapia
Feminino
Fibrose
Seguimentos
Seres Humanos
Leucemia Linfocítica Crônica de Células B/sangue
Leucemia Linfocítica Crônica de Células B/diagnóstico
Modelos Logísticos
Masculino
Meia-Idade
Análise Multivariada
Prognóstico
Reticulina/metabolismo
Estudos Retrospectivos
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Reticulin)
[Em] Mês de entrada:1708
[Cu] Atualização por classe:170804
[Lr] Data última revisão:
170804
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170425
[St] Status:MEDLINE
[do] DOI:10.1007/s00277-017-2997-7


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[PMID]:28274535
[Au] Autor:Ozawa Y; Yamamoto H; Yasuo M; Takahashi H; Tateishi K; Ushiki A; Kawakami S; Fujinaga Y; Asaka S; Sano K; Takayama H; Imamura H; Hanaoka M
[Ad] Endereço:First Department of Internal Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto 390-8621, Japan. Electronic address: 13mh008h@shinshu-u.ac.jp.
[Ti] Título:Two patients with TAFRO syndrome exhibiting strikingly similar anterior mediastinal lesions with predominantly fat attenuation on chest computed tomography.
[So] Source:Respir Investig;55(2):176-180, 2017 Mar.
[Is] ISSN:2212-5353
[Cp] País de publicação:Netherlands
[La] Idioma:eng
[Ab] Resumo:We herein report on two middle-aged men with TAFRO (thrombocytopenia, anasarca, fever, reticulin fibrosis or renal failure, and organomegaly) syndrome, a unique clinicopathological variant of multicentric Castleman׳s disease recently proposed in Japan. Strikingly similar anterior mediastinal fat swellings with soft tissue density were observed in the patients on chest computed tomography. In TAFRO syndrome, bilateral pleural effusion and slight lymph node swelling are common in the thoracic region; however, anterior mediastinal lesions have not been previously observed. Although the mechanisms of anterior mediastinal lesions have not been defined, these lesions seem to have a close relationship with TAFRO syndrome.
[Mh] Termos MeSH primário: Edema/diagnóstico por imagem
Febre/diagnóstico por imagem
Mediastino
Radiografia Torácica
Trombocitopenia/diagnóstico por imagem
Tomografia Computadorizada por Raios X
[Mh] Termos MeSH secundário: Tecido Adiposo/diagnóstico por imagem
Tecido Adiposo/patologia
Adulto
Edema/patologia
Febre/patologia
Fibrose/diagnóstico por imagem
Fibrose/patologia
Seres Humanos
Linfonodos/diagnóstico por imagem
Linfonodos/patologia
Masculino
Mediastino/patologia
Meia-Idade
Síndrome POEMS/diagnóstico por imagem
Síndrome POEMS/patologia
Insuficiência Renal/diagnóstico por imagem
Insuficiência Renal/patologia
Reticulina
Síndrome
Trombocitopenia/patologia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Reticulin)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170919
[Lr] Data última revisão:
170919
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170310
[St] Status:MEDLINE


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[PMID]:28211153
[Au] Autor:Mudireddy M; Barraco D; Hanson CA; Pardanani A; Gangat N; Tefferi A
[Ad] Endereço:Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota.
[Ti] Título:The prognostic relevance of serum lactate dehydrogenase and mild bone marrow reticulin fibrosis in essential thrombocythemia.
[So] Source:Am J Hematol;92(5):454-459, 2017 May.
[Is] ISSN:1096-8652
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:The 2016 World Health Organization (WHO) diagnostic criteria for myeloproliferative neoplasms (MPN) underscore the prognostically-relevant distinction between essential thrombocythemia (ET) and prefibrotic primary myelofibrosis (pre-PMF). In addition, leukocytosis has been identified as an important prognostic marker in otherwise WHO-defined ET. However, controversy remains regarding the objectivity of morphologic criteria in distinguishing ET from pre-PMF and the precise prognostic cutoff values for leukocytosis. Serum lactate dehydrogenase (LDH) level might be a biologically more accurate measure of leukocyte turnover and a more sensitive marker of pre-PMF, in otherwise WHO-defined ET. In the current study of 183 consecutive patients with WHO-defined ET, the presence of grade 1 bone marrow (BM) fibrosis did not affect presenting clinical or laboratory features; in contrast, increased serum LDH at diagnosis was associated with leukocytosis (p = .002), thrombocytosis (p < .001), palpable splenomegaly (p = .03) and higher international prognostic score (IPSET) (p = .002); serum LDH did not correlate with BM fibrosis, JAK2/CALR/MPL or TET2/ASXL1 mutations. In univariate analysis, risk factors for survival included age ≥60 years (p = .002; HR 10.2, 95% CI 2.3-44.6), male sex (p = .02; HR 3.2, 95% CI 1.2-8.2), leukocyte count ≥15 × 10 /L (p = .007; HR 4.7, 95% CI 1.5-14.6), and increased serum LDH (p = .002; HR 3.7, 95% CI 1.5-9.1), but not BM fibrosis (p = .17). In multivariable analysis, age, sex and serum LDH remained significant; serum LDH also remained significant, in the context of IPSET (p = .003) and in patients with leukocytosis (p = .003). We conclude that serum LDH level carries an independent prognostic value for survival in ET and might represent a biologically more accurate surrogate for leukocytosis.
[Mh] Termos MeSH primário: Lactato Desidrogenases/sangue
Mielofibrose Primária/patologia
Trombocitemia Essencial/patologia
[Mh] Termos MeSH secundário: Idoso
Idoso de 80 Anos ou mais
Biomarcadores
Feminino
Seres Humanos
Leucocitose/enzimologia
Masculino
Meia-Idade
Prognóstico
Reticulina
Fatores de Risco
Trombocitemia Essencial/enzimologia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Biomarkers); 0 (Reticulin); EC 1.1.- (Lactate Dehydrogenases)
[Em] Mês de entrada:1706
[Cu] Atualização por classe:170613
[Lr] Data última revisão:
170613
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170218
[St] Status:MEDLINE
[do] DOI:10.1002/ajh.24689


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[PMID]:28006767
[Au] Autor:Brynes RK; Wong RS; Thein MM; Bakshi KK; Burgess P; Theodore D; Orazi A
[Ad] Endereço:Department of Pathology, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA.
[Ti] Título:A 2-Year, Longitudinal, Prospective Study of the Effects of Eltrombopag on Bone Marrow in Patients with Chronic Immune Thrombocytopenia.
[So] Source:Acta Haematol;137(2):66-72, 2017.
[Is] ISSN:1421-9662
[Cp] País de publicação:Switzerland
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: The long-term effects of eltrombopag on bone marrow (BM) reticulin and/or collagen deposition in previously treated adults with chronic immune thrombocytopenia (ITP) were assessed. METHODS: Three BM biopsies were collected at baseline and after 1 and 2 years of eltrombopag treatment. Specimens were centrally processed, stained for reticulin and collagen, independently reviewed by 2 hematopathologists, and rated according to the European Consensus 0-3 scale of marrow fibrosis (MF). RESULTS: Of 162 patients enrolled, 93 completed all 3 protocol-specified BM biopsies. All patients with a baseline assessment were negative for collagen. Of 159 patients assessed at baseline, 150 (94%) had normal reticulin (MF-0) and 9 (6%) had minimally increased reticulin (MF-1). After 2 years, 83/93 patients (89%) with BM biopsies had MF-0, 10 (11%) had MF-1, and none had MF-2 or MF-3. Five out of 127 patients (4%) at 1 year and 1 out of 93 (1%) at 2 years had collagen deposition. None of the patients had clinical symptoms typical of BM dysfunction or abnormalities of clinical concern based on white blood cell count or peripheral blood smear. CONCLUSION: For most patients with chronic ITP, eltrombopag is not associated with clinically relevant increases in BM reticulin or collagen formation.
[Mh] Termos MeSH primário: Benzoatos/administração & dosagem
Medula Óssea
Colágeno/metabolismo
Hidrazinas/administração & dosagem
Púrpura Trombocitopênica Idiopática
Pirazóis/administração & dosagem
Reticulina/metabolismo
[Mh] Termos MeSH secundário: Adolescente
Adulto
Idoso
Idoso de 80 Anos ou mais
Biópsia
Medula Óssea/metabolismo
Medula Óssea/patologia
Doença Crônica
Feminino
Seres Humanos
Masculino
Meia-Idade
Estudos Prospectivos
Púrpura Trombocitopênica Idiopática/tratamento farmacológico
Púrpura Trombocitopênica Idiopática/metabolismo
Púrpura Trombocitopênica Idiopática/patologia
[Pt] Tipo de publicação:CLINICAL TRIAL, PHASE IV; JOURNAL ARTICLE; MULTICENTER STUDY
[Nm] Nome de substância:
0 (Benzoates); 0 (Hydrazines); 0 (Pyrazoles); 0 (Reticulin); 9007-34-5 (Collagen); S56D65XJ9G (eltrombopag)
[Em] Mês de entrada:1703
[Cu] Atualização por classe:170317
[Lr] Data última revisão:
170317
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161223
[St] Status:MEDLINE
[do] DOI:10.1159/000452992


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[PMID]:27795511
[Au] Autor:Masaki Y; Kawabata H; Takai K; Tsukamoto N; Fujimoto S; Ishigaki Y; Kurose N; Kojima M; Nakamura S; Kinoshita T; Aoki S; Japanese TAFRO syndrome research team
[Ad] Endereço:Hematology and Immunology, Medicine, Kanazawa Medical University.
[Ti] Título:Proposed diagnostic criteria, disease severity classification, and treatment strategy for a novel disorder; TAFRO syndrome.
[So] Source:Rinsho Ketsueki;57(10):2029-2037, 2016.
[Is] ISSN:0485-1439
[Cp] País de publicação:Japan
[La] Idioma:jpn
[Ab] Resumo:TAFRO syndrome is a systemic inflammatory disorder manifesting as thrombocytopenia; anasarca including pleural effusion and ascites; fever; renal insufficiency; and organomegaly including hepatosplenomegaly and lymphadenopathy. Its onset may be acute or sub-acute, but its etiology remains unknown. Although several clinical and pathological characteristics of TAFRO syndrome resemble those of Castleman's disease, other specific features can differentiate between the two. Some patients have been successfully treated with glucocorticoids and/or immunosuppressants including cyclosporin A, tocilizumab and rituximab, whereas others are refractory to treatment, eventually succumbing to the disease. Early and reliable diagnoses and early treatments with appropriate agents are essential to enhancing patient survival. The 2015 updated diagnostic criteria, disease severity classification and treatment strategy for TAFRO syndrome, as formulated by Japanese research teams, are presented herein. Furthermore, clinicopathological data on 28 patients with this condition and similar symptoms (e.g., MCD with serositis and thrombocytopenia) were analyzed retrospectively.
[Mh] Termos MeSH primário: Edema
Febre
Trombocitopenia
[Mh] Termos MeSH secundário: Edema/complicações
Edema/diagnóstico
Edema/terapia
Febre/complicações
Febre/diagnóstico
Febre/terapia
Fibrose/complicações
Fibrose/diagnóstico
Fibrose/terapia
Seres Humanos
Reticulina/química
Índice de Gravidade de Doença
Trombocitopenia/complicações
Trombocitopenia/diagnóstico
Trombocitopenia/terapia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Reticulin)
[Em] Mês de entrada:1702
[Cu] Atualização por classe:171019
[Lr] Data última revisão:
171019
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161101
[St] Status:MEDLINE


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[PMID]:27795506
[Au] Autor:Iwanaga N; Harada K; Tsuji Y; Kawahara C; Kurohama K; Izumi Y; Yoshida S; Fujikawa K; Ito M; Kawakami A; Migita K
[Ad] Endereço:Departments of General Internal Medicine and Rheumatology, Nagasaki Medical Center.
[Ti] Título:TAFRO syndrome with primary Sjogren's syndrome.
[So] Source:Nihon Rinsho Meneki Gakkai Kaishi;39(5):478-484, 2016.
[Is] ISSN:1349-7413
[Cp] País de publicação:Japan
[La] Idioma:jpn
[Ab] Resumo:A 25-year-old woman diagnosed 1 year earlier with Primary Sjogren's syndrome was admitted to a nearby hospital with fever of unknown origin. Examination revealed anasarca, systemic lymphadenopathy, hepatosplenomegaly and high C-reactive protein level. The patient's symptoms were initially suspected to be caused by severe bacterial infection with Sjogren's syndrome flare. She was given antibiotics and prednisolone (PSL) at 50 mg/day. However, the patient developed anemia and thrombocytopenia and was transferred to our hospital for further care. Histological examination of the right inguinal lymph node showed neutrophilic infiltration. Bone marrow aspiration revealed a normocellular marrow with increased megakaryocytes and mild reticulin fiber hyperplasia. After initiation of minocycline hydrochloride, the patient's symptoms improved. However, as PSL was tapered, her symptoms worsened. The patient's clinical symptoms and laboratory data improved again with initiation of intravenous steroid pulse therapy and cyclosporine. TAFRO syndrome is characterized by a constellation of symptoms: Thrombocytopenia, Anasarca, Fever, Reticulin fibrosis and Organomegaly. Although histological findings of the lymph node in this case differed from previous reports, the patient's other symptoms and clinical course were similar to TAFRO syndrome. TAFRO syndrome can occur with several diseases, including infection, rheumatic disease and malignancies. We report a case in which infection might have triggered TAFRO syndrome.
[Mh] Termos MeSH primário: Edema/etiologia
Febre/etiologia
Fibrose/etiologia
Síndrome POEMS/etiologia
Reticulina
Síndrome de Sjogren/complicações
Trombocitopenia/etiologia
[Mh] Termos MeSH secundário: Adulto
Infecções Bacterianas
Medula Óssea/patologia
Ciclosporina/uso terapêutico
Edema/tratamento farmacológico
Feminino
Febre/tratamento farmacológico
Fibrose/tratamento farmacológico
Seres Humanos
Linfonodos/patologia
Linfadenite/etiologia
Linfadenite/microbiologia
Linfadenite/patologia
Síndrome POEMS/tratamento farmacológico
Síndrome de Sjogren/patologia
Síndrome
Trombocitopenia/tratamento farmacológico
Resultado do Tratamento
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Reticulin); 83HN0GTJ6D (Cyclosporine)
[Em] Mês de entrada:1703
[Cu] Atualização por classe:171015
[Lr] Data última revisão:
171015
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161101
[St] Status:MEDLINE


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[PMID]:27415013
[Au] Autor:Tadeo I; Berbegall AP; Castel V; García-Miguel P; Callaghan R; Påhlman S; Navarro S; Noguera R
[Ad] Endereço:Department of Pathology, Medical School, University of Valencia - INCLIVA, Avda. Blasco Ibañez 15, 46010 Valencia, Spain.
[Ti] Título:Extracellular matrix composition defines an ultra-high-risk group of neuroblastoma within the high-risk patient cohort.
[So] Source:Br J Cancer;115(4):480-9, 2016 Aug 09.
[Is] ISSN:1532-1827
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Although survival for neuroblastoma patients has dramatically improved in recent years, a substantial number of children in the high-risk subgroup still die. METHODS: We aimed to define a subgroup of ultra-high-risk patients from within the high-risk cohort. We used advanced morphometric approaches to quantify and characterise blood vessels, reticulin fibre networks, collagen type I bundles, elastic fibres and glycosaminoglycans in 102 high-risk neuroblastomas specimens. The Kaplan-Meier method was used to correlate the analysed elements with survival. RESULTS: The organisation of blood vessels and reticulin fibres in neuroblastic tumours defined an ultra-high-risk patient subgroup with 5-year survival rate <15%. Specifically, tumours with irregularly shaped blood vessels, large sinusoid-like vessels, smaller and tortuous venules and arterioles and with large areas of reticulin fibres forming large, crosslinking, branching and haphazardly arranged networks were linked to the ultra-high-risk phenotype. CONCLUSIONS: We demonstrate that quantification of tumour stroma components by morphometric techniques has the potential to improve risk stratification of neuroblastoma patients.
[Mh] Termos MeSH primário: Neoplasias Encefálicas/patologia
Matriz Extracelular/patologia
Neuroblastoma/patologia
[Mh] Termos MeSH secundário: Vasos Sanguíneos/patologia
Neoplasias Encefálicas/mortalidade
Colágeno Tipo I/metabolismo
Tecido Elástico/metabolismo
Tecido Elástico/patologia
Matriz Extracelular/metabolismo
Glicosaminoglicanos/metabolismo
Seres Humanos
Lactente
Estimativa de Kaplan-Meier
Neuroblastoma/metabolismo
Neuroblastoma/mortalidade
Prognóstico
Reticulina/metabolismo
Risco
Medição de Risco
Taxa de Sobrevida
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Collagen Type I); 0 (Glycosaminoglycans); 0 (Reticulin)
[Em] Mês de entrada:1705
[Cu] Atualização por classe:170809
[Lr] Data última revisão:
170809
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160715
[St] Status:MEDLINE
[do] DOI:10.1038/bjc.2016.210


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[PMID]:27285854
[Au] Autor:Aqil B; Punia JN; Curry CV; Marcogliese AN; Elghetany MT
[Ad] Endereço:Department of Pathology & Immunology, Texas Children's Hospital and Baylor College of Medicine, Houston, TX, USA.
[Ti] Título:Are micromegakaryocytes specific for refractory cytopenia of childhood (RCC)? A study of 38 pediatric patients with thrombocytopenia unrelated to RCC.
[So] Source:Leuk Res;47:84-7, 2016 Aug.
[Is] ISSN:1873-5835
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Micromegakaryocytes (microMKs) are considered the most reliable dysplastic feature for myelodysplastic syndrome (MDS), particularly refractory cytopenia of childhood (RCC); there is no minimal threshold for the diagnosis of RCC. Since most RCC patients present with thrombocytopenia, the presence of microMKs should raise concern for MDS/RCC. This study attempted to investigate the prevalence of microMKs and associated marrow fibrosis in patients with thrombocytopenia unrelated to MDS/RCC and the need for establishing a threshold for microMKs for the diagnosis of MDS/RCC. DESIGN: Bone marrow biopsies of pediatric patients with thrombocytopenia unrelated to RCC were examined for microMKs and fibrosis by CD61 immunohistochemical and reticulin stains respectively. RESULT: Thirty eight patients (1-18 years old) were included: 33 immune thrombocytopenia (ITP), 3 chronic thrombocytopenia, and 2 inherited macrothrombocytopenia. Fourteen cases (37%) had microMKs; four cases showed increased marrow fibrosis associated with microMKs (two had ITP and two had macrothrombocytopenia). All patients are alive and none developed MDS (follow up: 3months to 4 years). CONCLUSION: MicroMKs can be seen in pediatric patients with thrombocytopenia unrelated to RCC. Hence the mere presence of microMKs is insufficient for the diagnosis of RCC in the pediatric population, and a quantitative threshold needs to be established.
[Mh] Termos MeSH primário: Megacariócitos/patologia
Síndromes Mielodisplásicas/diagnóstico
Trombocitopenia/patologia
[Mh] Termos MeSH secundário: Adolescente
Exame de Medula Óssea
Criança
Pré-Escolar
Diagnóstico Diferencial
Seres Humanos
Lactente
Integrina beta3/análise
Síndromes Mielodisplásicas/patologia
Mielofibrose Primária
Reticulina/análise
Trombocitopenia/complicações
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (ITGB3 protein, human); 0 (Integrin beta3); 0 (Reticulin)
[Em] Mês de entrada:1707
[Cu] Atualização por classe:170717
[Lr] Data última revisão:
170717
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160611
[St] Status:MEDLINE



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