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[PMID]:29429161
[Au] Autor:Li L; Duan XJ; Sun Y; Lu Y; Xu HY; Wang QZ; Wang HY
[Ad] Endereço:Department of Pathology, Fuwai Hospital, Chinese Academy of Medical Sciences, Beijing 100037, China.
[Ti] Título:[Classification of cardiac amyloidosis: an immunohistochemical analysis].
[So] Source:Zhonghua Bing Li Xue Za Zhi;47(2):105-109, 2018 Feb 08.
[Is] ISSN:0529-5807
[Cp] País de publicação:China
[La] Idioma:chi
[Ab] Resumo:To evaluate the sensitivity and specificity of immunohistochemistry (IHC) in the classification of cardiac amyloidosis on endomyocardial biopsy (EMB) and heart allograft. Twenty cardiac tissues from 19 patients at Fuwai Hospital from January, 1990 to April, 2017 with histopathologic features of amyloidosis and Congo red staining positivity were included. IHC was performed with monoclonal antibodies against AA amyloid and polyclonal antibodies against transthyretin (ATTR), λ-light chain (AL-λ), κ-light chain (AL-κ), ApoAâ… , ApoAâ…¡, ApoA â…£ and ß(2)-microglobin. The extent of interstitial staining was evaluated by light microscopy, and three patterns were recognized; these included diffuse pericellular pattern, discrete pericellular pattern, and nodular pattern. Two patterns of vascular deposition were also noted, including arterial pattern and venous pattern. Endocardial involvement was also assessed and recorded. Nineteen cases were divided into three groups according to the pattern of proteins expression in specimens. The first group (5 cases) only showed single protein expression on EMB. The second group (6 cases) showed more than one protein expression, but one of them was intensely stained or any staining of any protein together with ApoA â…£ co-staining. The third group (8 cases) also showed more than one protein expression and all of them had intense staining. Amyloid deposits were successfully subtyped as AL-λ, ATTR, AL-κ and ApoAâ… by IHC in the former two groups with the sensitivity of 11/19. In the third group, amyloid deposits could not be subtyped by immunohistochemistry due to their poor specificity. The pericellular pattern tended to favor AL over ATTR amyloidosis and vascular deposition tended to favor ATTR. Amyloid deposits can be reliably subtyped in diagnostic cardiac specimens using IHC. The co-deposition of chaperon proteins, the distribution of amyloid proteins and clinical features are also auxiliary to subtype cardiac amyloidosis.
[Mh] Termos MeSH primário: Amiloidose/patologia
Cardiomiopatias/patologia
[Mh] Termos MeSH secundário: Amiloide/análise
Neuropatias Amiloides Familiares/patologia
Anticorpos Monoclonais/análise
Apolipoproteína A-I/análise
Apolipoproteínas A/análise
Biópsia
Seres Humanos
Cadeias kappa de Imunoglobulina/análise
Cadeias lambda de Imunoglobulina/análise
Imuno-Histoquímica
Placa Amiloide/patologia
Sensibilidade e Especificidade
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (APOA1 protein, human); 0 (Amyloid); 0 (Antibodies, Monoclonal); 0 (Apolipoprotein A-I); 0 (Apolipoproteins A); 0 (Immunoglobulin kappa-Chains); 0 (Immunoglobulin lambda-Chains); 0 (apolipoprotein A-IV)
[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.0529-5807.2018.02.005


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[PMID]:29202938
[Au] Autor:Geller HI; Singh A; Mirto TM; Padera R; Mitchell R; Laubach JP; Falk RH
[Ad] Endereço:Cardiac Amyloidosis Program, Brigham and Women's Hospital, Harvard Medical School, Boston, MA.
[Ti] Título:Prevalence of Monoclonal Gammopathy in Wild-Type Transthyretin Amyloidosis.
[So] Source:Mayo Clin Proc;92(12):1800-1805, 2017 Dec.
[Is] ISSN:1942-5546
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:OBJECTIVE: To evaluate the prevalence of monoclonal gammopathy (MG) in patients with wild-type transthyretin amyloidosis (ATTRwt) (formerly known as senile amyloidosis). PATIENTS AND METHODS: We retrospectively analyzed the serum protein electrophoresis and serum immunofixation results, free light chain (FLC) levels, and renal function of 113 consecutive patients with ATTRwt seen at the Brigham and Women's Hospital's Cardiac Amyloidosis Program between February 21, 2006, and November 9, 2016. Monoclonal gammopathy was defined as a monoclonal protein present in the serum. Light chain MG was defined as an abnormal serum FLC κ/λ ratio with an elevated FLC level in the absence of a monoclonal protein. In patients with renal dysfunction, the renal FLC reference range was used. RESULTS: The mean age of the population was 75 years, 3 of the 113 patients (3%) were female, and 110 (97%) were white. Monoclonal gammopathy was present in 26 patients (23%), 24 of whom had monoclonal protein present and 2 others who met criteria for light chain MG. Most clones (12 of 20 [60%]) were λ restricted. Another 7 patients had an abnormal FLC κ/λ ratio in the setting of renal dysfunction. CONCLUSION: In this study, MG was present in 23% of patients with ATTRwt. The finding of MG or an abnormal FLC κ/λ ratio in an elderly man may cause diagnostic confusion during subtyping of amyloidosis. A high degree of clinical suspicion for ATTRwt and precise tissue typing using mass spectrometry may overcome such diagnostic challenges.
[Mh] Termos MeSH primário: Neuropatias Amiloides Familiares/imunologia
Gamopatia Monoclonal de Significância Indeterminada/diagnóstico
[Mh] Termos MeSH secundário: Idoso
Idoso de 80 Anos ou mais
Feminino
Seres Humanos
Cadeias kappa de Imunoglobulina/sangue
Cadeias lambda de Imunoglobulina/sangue
Masculino
Meia-Idade
Prevalência
Estudos Retrospectivos
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Immunoglobulin kappa-Chains); 0 (Immunoglobulin lambda-Chains)
[Em] Mês de entrada:1712
[Cu] Atualização por classe:171212
[Lr] Data última revisão:
171212
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:171206
[St] Status:MEDLINE


  3 / 3101 MEDLINE  
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[PMID]:28957409
[Au] Autor:Moreau JM; Cen S; Berger A; Furlonger C; Paige CJ
[Ad] Endereço:Princess Margaret Cancer Centre, University Health Network, Toronto, Canada.
[Ti] Título:Bone marrow basophils provide survival signals to immature B cells in vitro but are dispensable in vivo.
[So] Source:PLoS One;12(9):e0185509, 2017.
[Is] ISSN:1932-6203
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Immature B cells are the first B cell progenitors to express a fully formed B cell receptor and are therefore subject to extensive selection processes that act to mitigate the emergence of autoreactive clones. While it is well appreciated that most B cell generation in the bone marrow is highly dependent on access to molecules present in the local milieu, the existence of extrinsically provided factors that modulate immature B cell biology is ambiguous. Nonetheless, a population of CD49b+CD90lo cells has demonstrated in vitro potential to promote immature B cell survival. Using a mouse basophil reporter strain we confirmed the identity of these CD49b+CD90lo supportive cells as basophils. However, analysis of bone marrow B cell populations following lineage specific basophil depletion demonstrates that basophils do not have a significant role in vivo in modulating immature B cell biology during steady-state conditions.
[Mh] Termos MeSH primário: Basófilos/citologia
Células da Medula Óssea/citologia
Células Precursoras de Linfócitos B/citologia
[Mh] Termos MeSH secundário: Animais
Basófilos/metabolismo
Células da Medula Óssea/metabolismo
Linhagem da Célula
Sobrevivência Celular
Técnicas de Cocultura
Citoproteção
Feminino
Proteínas de Homeodomínio/metabolismo
Cadeias lambda de Imunoglobulina/metabolismo
Integrina alfa2/metabolismo
Contagem de Linfócitos
Camundongos
Células Precursoras de Linfócitos B/metabolismo
Antígenos Thy-1/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Homeodomain Proteins); 0 (Immunoglobulin lambda-Chains); 0 (Integrin alpha2); 0 (Thy-1 Antigens); 128559-51-3 (RAG-1 protein)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171116
[Lr] Data última revisão:
171116
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170929
[St] Status:MEDLINE
[do] DOI:10.1371/journal.pone.0185509


  4 / 3101 MEDLINE  
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[PMID]:28595732
[Au] Autor:Akasaka T; Kishimori C; Fukutsuka K; Nakagawa M; Takeoka K; Hayashida M; Honjo G; Ohno H
[Ad] Endereço:Department of Hematology, Tenri Hospital, Japan. Electronic address: akasaka@tenriyorozu.jp.
[Ti] Título:The novel double-hit, t(8;22)(q24;q11)/MYC-IGL and t(14;15)(q32;q24)/IGH-BCL2A1, in diffuse large B-cell lymphoma.
[So] Source:Cancer Genet;214-215:26-31, 2017 Aug.
[Is] ISSN:2210-7762
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:An 82-year-old woman presented with generalized lymphadenopathy and skin involvement. Lymph node biopsy revealed diffuse large B-cell lymphoma with a high proliferation index. G-banding and fluorescence in situ hybridization showed a hypertetraploid karyotype with two copies of t(8;22)(q24;q11), generating the fusion of MYC and the immunoglobulin λ chain gene (IGL), and two copies of the novel immunoglobulin heavy chain gene (IGH) translocation, t(14;15)(q32;q24). A long-distance inverse polymerase chain reaction (PCR) using nested primer combinations designed for each constant gene of IGH showed that Cγ4 was juxtaposed to the downstream sequence of the BCL2A1 (BCL2-related protein A1) gene through the Sγ4 switch region. As a result of t(14;15)(q32;q24), BCL2A1 and IGH Sγ4-Cγ4 were aligned in the same transcriptional orientation at a distance of 64 kb. Reverse transcriptase-mediated PCR showed high BCL2A1 mRNA levels in a lymphoma specimen. Since BCL2A1, mapped at 15q24.3 or 15q25.1, encodes a protein that is an anti-apoptotic member of the BCL2 protein family, we herein described the novel double-hit, t(8;22)(q24;q11)/MYC-IGL and t(14;15)(q32;q24)/IGH-BCL2A1, in which BCL2A1 is considered to play a role equivalent to that of BCL2 in the most frequent double-hit, MYC/BCL2.
[Mh] Termos MeSH primário: Cadeias Pesadas de Imunoglobulinas/genética
Cadeias lambda de Imunoglobulina/genética
Linfoma Difuso de Grandes Células B/genética
Antígenos de Histocompatibilidade Menor/genética
Proteínas Proto-Oncogênicas c-bcl-2/genética
Proteínas Proto-Oncogênicas c-myc/genética
Translocação Genética
[Mh] Termos MeSH secundário: Idoso de 80 Anos ou mais
Cromossomos Humanos Par 14
Cromossomos Humanos Par 15
Cromossomos Humanos Par 22
Cromossomos Humanos Par 8
Feminino
Seres Humanos
Hibridização in Situ Fluorescente
Cariótipo
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (BCL2-related protein A1); 0 (Immunoglobulin Heavy Chains); 0 (Immunoglobulin lambda-Chains); 0 (MYC protein, human); 0 (Minor Histocompatibility Antigens); 0 (Proto-Oncogene Proteins c-bcl-2); 0 (Proto-Oncogene Proteins c-myc)
[Em] Mês de entrada:1708
[Cu] Atualização por classe:170815
[Lr] Data última revisão:
170815
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170610
[St] Status:MEDLINE


  5 / 3101 MEDLINE  
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[PMID]:28575180
[Au] Autor:Messiaen AS; De Sloovere MMW; Claus PE; Vercammen M; Van Hoovels L; Heylen O; Debrabandere J; Vanpoucke H; De Smet D
[Ad] Endereço:From the Department of Laboratory Medicine, AZ Delta Roeselare-Menen, Roeselare, Belgium.
[Ti] Título:Performance Evaluation of Serum Free Light Chain Analysis: Nephelometry vs Turbidimetry, Monoclonal vs Polyclonal Reagents.
[So] Source:Am J Clin Pathol;147(6):611-622, 2017 Jun 01.
[Is] ISSN:1943-7722
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Objectives: Free light chain (FLC) measurement gained a lot of interest for diagnostic workup of monoclonal gammopathy. Methods: We evaluated the performance of turbidimetric polyclonal Freelite (The Binding Site, Birmingham, UK) assays on Cobas 6000 (Roche Diagnostics, Rotkreuz, Switzerland) and nephelometric monoclonal N Latex (Siemens Healthcare Diagnostics, Marburg, Germany) assays on BN ProSpec (Dade Behring, Deerfield, IL) vs established nephelometric Freelite assays on BN ProSpec. Results: Analytical performance was acceptable. Method comparison (n = 118) showed significant proportional FLC differences for N Latex assays. However, good correlation and clinical concordance were shown. Recovery study in the low concentration range demonstrated consistent over- and underrecovery for Freelite reagents, hampering future research on prognostic value of suppressed noninvolved FLC. Antigen excess detection was successful for κ FLC in three-fourths of cases with Freelite reagents and in all cases with N Latex reagents. However, the latter resulted in underestimated κ FLC concentrations. Conclusions: FLC analysis requires continuous awareness of analytical limitations. Monitoring of disease response requires FLC analysis on the same platform using the same reagents.
[Mh] Termos MeSH primário: Cadeias Leves de Imunoglobulina/sangue
Cadeias kappa de Imunoglobulina/sangue
Cadeias lambda de Imunoglobulina/sangue
Nefelometria e Turbidimetria/métodos
Paraproteinemias/diagnóstico
[Mh] Termos MeSH secundário: Anticorpos Monoclonais/imunologia
Seres Humanos
Cadeias Leves de Imunoglobulina/imunologia
Cadeias kappa de Imunoglobulina/imunologia
Cadeias lambda de Imunoglobulina/imunologia
Paraproteinemias/imunologia
Prognóstico
Kit de Reagentes para Diagnóstico
Sensibilidade e Especificidade
[Pt] Tipo de publicação:EVALUATION STUDIES; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Antibodies, Monoclonal); 0 (Immunoglobulin Light Chains); 0 (Immunoglobulin kappa-Chains); 0 (Immunoglobulin lambda-Chains); 0 (Reagent Kits, Diagnostic)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171013
[Lr] Data última revisão:
171013
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170603
[St] Status:MEDLINE
[do] DOI:10.1093/ajcp/aqx037


  6 / 3101 MEDLINE  
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[PMID]:28367947
[Au] Autor:Kawakami N; Katsuyama Y; Hagiwara Y; Yoshida H; Kim K; Harada K
[Ad] Endereço:Department of Neurology, Shizuoka General Hospital.
[Ti] Título:A case of amyloid myopathy diagnosed during the treatment of myopathy associated with anti-signal recognition particle antibodies.
[So] Source:Rinsho Shinkeigaku;57(4):168-173, 2017 04 28.
[Is] ISSN:1882-0654
[Cp] País de publicação:Japan
[La] Idioma:jpn
[Ab] Resumo:A 78-year-old man presented with subacute progressive proximal weakness and dysphagia. A biopsy specimen from the left biceps femoris revealed evidence of necrotic and regenerating muscle fibers, but lymphocyte infiltration was not noted. The patient was diagnosed with necrotizing myopathy with anti-signal recognition particle (SRP) antibodies. Concomitant therapy with prednisolone and azathioprine caused the serum CK level to return to normal and it caused clinical manifestations to abate. One year later, however, muscle weakness worsened. Immunoelectrophoresis of serum revealed IgG M protein, and muscle pathology revealed amyloid deposits in numerous blood vessels and at the periphery of a few muscle fibers, and deposits stained positive for anti-λ light chain antibody. The patient was diagnosed with amyloid myopathy, and therapy for systemic amyloid light chain amyloidosis caused muscle weakness to diminish. Amyloidosis is believed to be the primary pathology in this case based on the patient's response to treatment reaction, but the significance of a case involving both amyloid myopathy and necrotizing myopathy warranted examination.
[Mh] Termos MeSH primário: Amiloidose/diagnóstico
Autoanticorpos/sangue
Doenças Musculares/diagnóstico
Partícula de Reconhecimento de Sinal/imunologia
[Mh] Termos MeSH secundário: Idoso
Amiloide/metabolismo
Amiloidose/tratamento farmacológico
Amiloidose/patologia
Biomarcadores/sangue
Biomarcadores/metabolismo
Glicoproteínas/sangue
Seres Humanos
Imunoglobulina G/sangue
Cadeias lambda de Imunoglobulina/metabolismo
Imagem por Ressonância Magnética
Masculino
Músculos/diagnóstico por imagem
Músculos/metabolismo
Músculos/patologia
Doenças Musculares/tratamento farmacológico
Doenças Musculares/patologia
Necrose
Resultado do Tratamento
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Amyloid); 0 (Autoantibodies); 0 (Biomarkers); 0 (Glycoproteins); 0 (Immunoglobulin G); 0 (Immunoglobulin lambda-Chains); 0 (Signal Recognition Particle); 0 (protein M (glycoprotein))
[Em] Mês de entrada:1706
[Cu] Atualização por classe:171012
[Lr] Data última revisão:
171012
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170404
[St] Status:MEDLINE
[do] DOI:10.5692/clinicalneurol.cn-000974


  7 / 3101 MEDLINE  
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[PMID]:28355725
[Au] Autor:An N; Chen SL; Li X
[Ti] Título:[A case report of immunoglonulin λ light chain amyloidosis combined with leukocyte cell-derived chemotaxin-2 amyloidosis].
[So] Source:Zhonghua Nei Ke Za Zhi;56(4):298-300, 2017 Apr 01.
[Is] ISSN:0578-1426
[Cp] País de publicação:China
[La] Idioma:chi
[Mh] Termos MeSH primário: Amiloidose/diagnóstico
Amiloidose/imunologia
Cadeias Leves de Imunoglobulina/imunologia
Cadeias lambda de Imunoglobulina
Leucócitos
[Mh] Termos MeSH secundário: Fatores Quimiotáticos
Seres Humanos
Masculino
Meia-Idade
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Chemotactic Factors); 0 (Immunoglobulin Light Chains); 0 (Immunoglobulin lambda-Chains)
[Em] Mês de entrada:1707
[Cu] Atualização por classe:170731
[Lr] Data última revisão:
170731
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170330
[St] Status:MEDLINE
[do] DOI:10.3760/cma.j.issn.0578-1426.2017.04.012


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[PMID]:28334621
[Au] Autor:Le Bras F; Molinier-Frenkel V; Guellich A; Dupuis J; Belhadj K; Guendouz S; Ayad K; Colombat M; Benhaiem N; Tissot CM; Hulin A; Jaccard A; Damy T
[Ad] Endereço:Lymphoid Malignancies Unit, Henri Mondor Hospital, Créteil, France; Paris XII University, UPEC, Créteil, France; GRC Amyloid Research Institute, IMRB-INSERM U955, and Mondor Amyloidosis Network, Créteil, France.
[Ti] Título:Sequential cyclophosphamide-bortezomib-dexamethasone unmasks the harmful cardiac effect of dexamethasone in primary light-chain cardiac amyloidosis.
[So] Source:Eur J Cancer;76:183-187, 2017 May.
[Is] ISSN:1879-0852
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Chemotherapy combining cyclophosphamide, bortezomib and dexamethasone is widely used in light-chain amyloidosis. The benefit is limited in patients with cardiac amyloidosis mainly because of adverse cardiac events. Retrospective analysis of our cohort showed that 39 patients died with 42% during the first month. A new escalation-sequential regimen was set to improve the outcomes. Nine newly-diagnosed patients were prospectively treated with close monitoring of serum N-terminal pro-brain natriuretic peptide, troponin-T and free light chains. The results show that corticoids may destabilise the heart through fluid retention. Thus, a sequential protocol may be a promising approach to treat these patients.
[Mh] Termos MeSH primário: Amiloidose/tratamento farmacológico
Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos
Arritmias Cardíacas/induzido quimicamente
Morte Súbita Cardíaca/etiologia
Dexametasona/efeitos adversos
Cardiopatias/tratamento farmacológico
Cadeias Leves de Imunoglobulina/sangue
Desequilíbrio Hidroeletrolítico/induzido quimicamente
[Mh] Termos MeSH secundário: Idoso
Idoso de 80 Anos ou mais
Amiloidose/sangue
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico
Bortezomib/administração & dosagem
Ciclofosfamida/administração & dosagem
Dexametasona/administração & dosagem
Feminino
Cardiopatias/sangue
Seres Humanos
Amiloidose de Cadeia Leve de Imunoglobulina
Cadeias kappa de Imunoglobulina/sangue
Cadeias lambda de Imunoglobulina/sangue
Masculino
Meia-Idade
Peptídeo Natriurético Encefálico/sangue
Fragmentos de Peptídeos/sangue
Estudos Retrospectivos
Troponina T/sangue
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Immunoglobulin Light Chains); 0 (Immunoglobulin kappa-Chains); 0 (Immunoglobulin lambda-Chains); 0 (Peptide Fragments); 0 (Troponin T); 0 (pro-brain natriuretic peptide (1-76)); 114471-18-0 (Natriuretic Peptide, Brain); 69G8BD63PP (Bortezomib); 7S5I7G3JQL (Dexamethasone); 8N3DW7272P (Cyclophosphamide)
[Em] Mês de entrada:1708
[Cu] Atualização por classe:171116
[Lr] Data última revisão:
171116
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170324
[St] Status:MEDLINE


  9 / 3101 MEDLINE  
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[PMID]:28202864
[Au] Autor:Inoue R; Fujigaki Y; Kobayashi K; Tamura Y; Ota T; Shibata S; Ishida T; Kondo F; Yamaguchi Y; Uchida S
[Ad] Endereço:Department of Internal Medicine, Teikyo University School of Medicine, Japan.
[Ti] Título:Clinical Presentation of Tubulointerstitial Nephritis Caused by Amyloid Light-chain Amyloidosis in a Patient with Sjögren's Syndrome.
[So] Source:Intern Med;56(4):419-423, 2017.
[Is] ISSN:1349-7235
[Cp] País de publicação:Japan
[La] Idioma:eng
[Ab] Resumo:We report a 70-year-old woman with Sjögren's syndrome who had severe renal dysfunction with mild proteinuria and elevated urinary low-molecular-weight proteins. Based on these clinical presentations, interstitial nephritis due to Sjögren's syndrome was strongly suspected. Unexpectedly, renal pathology revealed amyloid light-chain (AL) lambda-type depositions predominantly in the vasculatures with severe tubulointerstitial damage. Concentrated urine immunofixation was positive for Bence Jones lambda-type monoclonal proteins. Given the involvement in other organs, systemic AL amyloidosis was diagnosed. The patient underwent chemotherapy, but hemodialysis was ultimately instituted. It should be remembered that renal amyloidosis occurs as a clinical presentation of interstitial nephritis.
[Mh] Termos MeSH primário: Amiloidose/complicações
Nefrite Intersticial/etiologia
Síndrome de Sjogren/complicações
[Mh] Termos MeSH secundário: Idoso
Amiloide/metabolismo
Amiloidose/diagnóstico
Proteína de Bence Jones/urina
Biópsia
Feminino
Seres Humanos
Cadeias lambda de Imunoglobulina/urina
Rim/patologia
Nefrite Intersticial/patologia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Amyloid); 0 (Immunoglobulin lambda-Chains); 9006-99-9 (Bence Jones Protein)
[Em] Mês de entrada:1704
[Cu] Atualização por classe:170410
[Lr] Data última revisão:
170410
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170217
[St] Status:MEDLINE
[do] DOI:10.2169/internalmedicine.56.7548


  10 / 3101 MEDLINE  
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[PMID]:28195098
[Au] Autor:Ghodke KA; Patkar NV; Subramanian PG; Gujral S; Kadam PA; Tembhare PR
[Ad] Endereço:Department of Pathology, Hematopathology Laboratory, Tata Memorial Centre, Mumbai, Maharashtra, India.
[Ti] Título:Biclonal chronic lymphocytic leukemia: A study of two cases and review of literature.
[So] Source:Indian J Pathol Microbiol;60(1):84-86, 2017 Jan-Mar.
[Is] ISSN:0974-5130
[Cp] País de publicação:India
[La] Idioma:eng
[Ab] Resumo:Chronic lymphocytic leukemia (CLL) is a common, immunophenotypically well-defined mature B-cell neoplasm. Demonstration of more than 5000/µL CD5+ B-cell population with co-expression of CD23, weak expression of CD20, and one type of immunoglobin light chain (either kappa or lambda) is necessary for the diagnosis of CLL. However, CLL with two populations of B-cells expressing both kappa as well as lambda (biclonal) light chains are extremely rare and has not been reported from India. We report two cases of biclonal CLL presented with leukocytosis, typical morphological features, and distinct immunophenotype of CLL. These cases are also an example which suggests that careful attention to the morphology of the blood smear and the entire immunophenotype panel is a must and will aid the proper diagnosis as only light chain ratios can be misguiding.
[Mh] Termos MeSH primário: Linfócitos B/química
Linfócitos B/classificação
Cadeias kappa de Imunoglobulina/análise
Cadeias lambda de Imunoglobulina/análise
Leucemia Linfocítica Crônica de Células B/diagnóstico
Leucemia Linfocítica Crônica de Células B/patologia
[Mh] Termos MeSH secundário: Adulto
Idoso
Seres Humanos
Imunofenotipagem
Índia
Masculino
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
0 (Immunoglobulin kappa-Chains); 0 (Immunoglobulin lambda-Chains)
[Em] Mês de entrada:1703
[Cu] Atualização por classe:170313
[Lr] Data última revisão:
170313
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170215
[St] Status:MEDLINE
[do] DOI:10.4103/0377-4929.200019



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