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3.
Presse Med ; 48(7-8 Pt 1): 792-806, 2019.
Artigo em Francês | MEDLINE | ID: mdl-31447335

RESUMO

Chronic lymphoproliferative disorders should be classified according to the revised 2016 WHO classification. Biopsies are not mandatory for all chronic lymphoproliferative disorders as blood or bone marrow cytologroachical approach can be sufficient for some lymphoma entities. Diagnostic is based on a multidiscplinary approach taking into account clinical presentation, histopathological, cytological, immunophenotypical features (immunohistochemistry and Flow cytometry) and molecular pattern (translocation by FISH, Mutations landscape by NGS, and genomic abnormalities by CGH array). An important heterogeneity of clinical presentation and prognosis arises within the same lymphoma subtype. Clinical evolution is characterized by relapses, cytological progression and transformation into diffuse large B cell lymphoma, aggressive lymphoma or high-grade lymphomas.


Assuntos
Linfócitos B/patologia , Leucemia Linfocítica Crônica de Células B/classificação , Leucemia Linfocítica Crônica de Células B/patologia , Transtornos Linfoproliferativos/classificação , Transtornos Linfoproliferativos/patologia , Doença Crônica , Diagnóstico Diferencial , Humanos , Leucemia Linfocítica Crônica de Células B/diagnóstico , Transtornos Linfoproliferativos/diagnóstico , Síndrome
6.
Surg Pathol Clin ; 12(3): 719-731, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31352984

RESUMO

Technical advances in diagnostic modalities have led to the characterization of indolent lymphoid disorders similar to the in situ lesions described in epithelial malignancies. These early and indolent lymphoid lesions share clinicopathologic characteristics with well-characterized lymphoid malignancies such as chronic lymphocytic leukemia and follicular lymphoma. The in situ lesions have an indolent clinical course with only a minor subset shown to progress to frank malignancies. In addition to the in situ lesions, new indolent lymphoproliferative disorders have been recently characterized. Diagnosis and characterization of these indolent lesions is necessary to prevent overtreatment with aggressive therapeutic regimens.


Assuntos
Linfoma Folicular/patologia , Transtornos Linfoproliferativos/patologia , Linfócitos B/patologia , Diagnóstico Diferencial , Humanos , Imunofenotipagem/métodos , Leucemia Linfocítica Crônica de Células B/patologia , Linfocitose/patologia , Linfoma de Célula do Manto/patologia , Prognóstico
7.
J Clin Exp Hematop ; 59(2): 48-55, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31257345

RESUMO

In the current revised 4th edition of the World Health Organization (WHO) classification, 'other iatrogenic immunodeficiency-associated lymphoproliferative disorders (Oii-LPDs)' is listed in the last section in the chapter on immunodeficiency-associated lymphoproliferative disorders. Oii-LPDs cover a broad spectrum from benign lesions to lymphoma, and correspond to one of the subtypes in the WHO classification for immunocompetent patients.The WHO classification does not clearly indicate the histological subtype of this disease category; however, the framework of subtype classification is similar to the classification of post-transplant lymphoproliferative disorders, and recent studies have attempted to subcategorize Oii-LPDs that fit this unique disease type. In this review, we provide an overview of B-cell-type Oii-LPDs regarding their histopathology and immunophenotype, genetics and clinical behaviors.


Assuntos
Linfócitos B/patologia , Síndromes de Imunodeficiência/patologia , Imunossupressores/efeitos adversos , Transtornos Linfoproliferativos/patologia , Animais , Linfócitos B/efeitos dos fármacos , Humanos , Doença Iatrogênica/epidemiologia , Síndromes de Imunodeficiência/induzido quimicamente , Síndromes de Imunodeficiência/classificação , Transtornos Linfoproliferativos/induzido quimicamente , Transtornos Linfoproliferativos/classificação
8.
J Clin Exp Hematop ; 59(2): 56-63, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31257346

RESUMO

Other iatrogenic immunodeficiency-associated lymphoproliferative disorders (OIIA-LPDs) with a T- or NK-cell phenotype are markedly rare, with only a limited number of cases having been reported thus far. Methotrexate (MTX) is the most common agent used for OIIA-LPD patients, and 43 cases of MTX-associated T-LPDs (MTX T-LPDs) and five cases of MTX-associated NK/T-LPDs (MTX NK-LPDs) have been described. In addition to MTX T-LPDs and MTX NK/T-LPDs, T-LPD and NK/T-LPDs have been reported in patients receiving other immunosuppressive agents such as thiopurines, TNF antagonists, and cyclosporine. Hepatosplenic T-cell lymphoma (HSTL) is specifically associated with iatrogenic immunodeficiency, and 10% of HSTL cases develop in patients receiving thiopurines and/or TNF antagonists for inflammatory bowel disease (IBD). In this review, we focused on MTX T-LPD, MTX NK/T-LPD, and HSTL in patients with IBD. These T- and NK/T-cell associated OIIA-LPDs are the most common in daily medical practice.


Assuntos
Síndromes de Imunodeficiência/patologia , Transtornos Linfoproliferativos/patologia , Linfócitos T/patologia , Animais , Humanos , Doença Iatrogênica/epidemiologia , Síndromes de Imunodeficiência/induzido quimicamente , Imunossupressores/efeitos adversos , Imunossupressores/uso terapêutico , Doenças Inflamatórias Intestinais/tratamento farmacológico , Transtornos Linfoproliferativos/induzido quimicamente , Metotrexato/efeitos adversos , Metotrexato/uso terapêutico , Células T Matadoras Naturais/efeitos dos fármacos , Células T Matadoras Naturais/patologia , Linfócitos T/efeitos dos fármacos
9.
J Clin Exp Hematop ; 59(2): 72-92, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31257348

RESUMO

Other iatrogenic immunodeficiency-associated lymphoproliferative disorders (OIIA-LPD), a category of immunodeficiency-associated LPD according to the World Health Organization classification, is associated with immunosuppressive drugs (ISDs). Several factors, including autoimmune disease (AID) activity, Epstein-Barr virus (EBV) infection, ISD usage, and aging, influence the development of OIIA-LPD, resulting in complicated clinical courses and outcomes. Most OIIA-LPD develops in patients with rheumatoid arthritis using methotrexate (MTX-LPD). The management of MTX-LPD is based on the clinical course, i.e., with/without regression, with/without relapse/regrowth event (RRE), LPD subtype, and ISDs for AIDs after LPD development. There are three clinical courses after ISD withdrawal: regressive LPD without relapse/regrowth (R-G), regressive LPD with RRE (R/R-G), and persistent LPD (P-G). The majority of EBV+ diffuse large B-cell lymphomas are classified in R-G, whereas classic Hodgkin lymphoma is generally classified in R/R-G. Polymorphic LPD (P-LPD) in MTX-LPD develops with heterogeneous pathological features similar to monomorphic LPD. Chemotherapy for MTX-LPD is selected according to that for de novo LPD, although the strategy for aggressive P-LPD and non-specific LPD is not well established. The absolute lymphocyte count in the peripheral blood has been suggested as a candidate marker for MTX-LPD development and RRE. Several clinical issues, including correct diagnosis among overlapping clinicopathological features in MTX-LPD and clinical management of LPD by ISDs other than MTX, require further investigation.


Assuntos
Síndromes de Imunodeficiência/terapia , Transtornos Linfoproliferativos/terapia , Animais , Artrite Reumatoide/tratamento farmacológico , Gerenciamento Clínico , Humanos , Doença Iatrogênica/epidemiologia , Síndromes de Imunodeficiência/induzido quimicamente , Síndromes de Imunodeficiência/patologia , Imunossupressores/efeitos adversos , Imunossupressores/uso terapêutico , Transtornos Linfoproliferativos/induzido quimicamente , Transtornos Linfoproliferativos/patologia , Metotrexato/efeitos adversos , Metotrexato/uso terapêutico
10.
Diagn Pathol ; 14(1): 70, 2019 Jun 29.
Artigo em Inglês | MEDLINE | ID: mdl-31253137

RESUMO

BACKGROUND: Castleman disease (CD) is a lymphoproliferative disorder with an unknown etiology. The disease may be unicentric (UCD) or multicentric (MCD), and three histopathologic variants have been described: hyaline vascular (HV), plasma cell (PC), and mixed type. Extranodal CD is rare. Herein, we report a case of CD presenting as a tonsillar mass, which has not been documented in the literature. CASE PRESENTATION: The patient was a 32-year-old man. Laryngoscopy revealed tonsillar hypertrophy, and the patient underwent a low-temperature plasma tonsillectomy. Microscopic examination of permanent sections showed lymphoid follicular hyperplasia, a portion of which appeared to be a fusion of nodular hyperplasia (composed of lymphoid follicles of variable size and shape). These distinctive follicles with atrophic hyalinized germinal centers and a broad mantle zone of small lymphocytes formed concentric rings (so-called onion-skin arrangement). Medium-sized vessels and a plethora of capillaries were present in the center of the lymphatic follicles, mantle zones, and interfollicular areas. A characteristic lollipop appearance was also observed due to the onion-skin arrangement of the expanded mantle zone lymphocytes with a vessel penetrating the germinal center. No aberrant lymphoid population was present based on CD3, CD5, CD20, CD79α, CD21, CD23, bcl-2, cyclin D1, and ki-67 immunostaining. Tests for human herpesvirus (HHV)-8 and Epstein Barr virus (EBV)-encoded small RNA (EBER) were negative. Therefore, a diagnosis of an HV variant UCD was rendered. The patient was treated by local excision without any other therapy based on the diagnosis. At the 7-month follow up, the patient had no recurrent symptoms or masses. CONCLUSION: We present an unusual case of a tonsil presenting hyaline vascular Castleman disease (HVCD). This study aims to highlight CD as a differential diagnosis that should be considered by otolaryngologists and pathologists for lymphoproliferative disorders of the tonsil.


Assuntos
Hiperplasia do Linfonodo Gigante/diagnóstico , Hialina/metabolismo , Transtornos Linfoproliferativos/diagnóstico , Adulto , Hiperplasia do Linfonodo Gigante/patologia , Diagnóstico Diferencial , Centro Germinativo/patologia , Humanos , Imuno-Histoquímica , Linfonodos/patologia , Linfócitos/patologia , Tecido Linfoide/patologia , Transtornos Linfoproliferativos/patologia , Masculino , Tonsila Palatina/patologia
11.
Rev. esp. patol ; 52(2): 130-135, abr.-jun. 2019. ilus
Artigo em Espanhol | IBECS | ID: ibc-182701

RESUMO

La histiocitosis de células de Langerhans (HCL) es una enfermedad caracterizada por la proliferación de células dendríticas CD1a+con compromiso orgánico único o difuso. La identificación de mutaciones génicas recurrentes ha confirmado la hipótesis de HCL como una verdadera neoplasia. La papulosis linfomatoide (PL) pertenece al espectro de los linfomas cutáneos primarios CD30+. La HCL ha sido descrita en asociación con otros trastornos linfoproliferativos, pero hasta la fecha, las lesiones constituidas por células de Langerhans (CL) han sido consideradas de carácter reactivo, relacionada con citocinas producidas por la interacción linfoma microambiente. Algunos autores designan estas lesiones «Langerhans cells like lesions». Presentamos el caso de una mujer de 28 años con HCL multisistémica y presencia simultánea de lesiones de PL con hiperplasia reactiva de CL


Langerhans cell histiocytosis (LCH) is a disease characterized by proliferation of CD1a+dendritic cells with local or diffuse organ compromise. The identification of recurrent gene mutations has confirmed the hypothesis of LCH as a true neoplasm. Lymphomatoid papulosis (LyP) belongs to the spectrum of CD30+primary cutaneous lymphomas. LCH has been described in association with other lymphoproliferative disorders. However, lesions constituted by Langerhans cells (LC) have been commonly considered reactive, related to cytokines produced by the lymphoma-microenvironment interaction. Some authors designate these lesions as "Langerhans cells-like lesions". We present the case of a 28-years-old woman with multisystem LCH and simultaneous PyL lesions with reactive LC hyperplasia


Assuntos
Humanos , Feminino , Adulto , Histiocitose de Células de Langerhans/patologia , Papulose Linfomatoide/patologia , Células Dendríticas/patologia , Transtornos Linfoproliferativos/patologia , Prednisona/uso terapêutico , Cladribina/uso terapêutico
12.
Blood ; 133(26): 2753-2764, 2019 06 27.
Artigo em Inglês | MEDLINE | ID: mdl-31064750

RESUMO

Patients with classic hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) typically have high levels of Epstein-Barr virus (EBV) DNA in T cells and/or natural killer (NK) cells in blood and skin lesions induced by sun exposure that are infiltrated with EBV-infected lymphocytes. HVLPD is very rare in the United States and Europe but more common in Asia and South America. The disease can progress to a systemic form that may result in fatal lymphoma. We report our 11-year experience with 16 HVLPD patients from the United States and England and found that whites were less likely to develop systemic EBV disease (1/10) than nonwhites (5/6). All (10/10) of the white patients were generally in good health at last follow-up, while two-thirds (4/6) of the nonwhite patients required hematopoietic stem cell transplantation. Nonwhite patients had later age of onset of HVLPD than white patients (median age, 8 vs 5 years) and higher levels of EBV DNA (median, 1 515 000 vs 250 000 copies/ml) and more often had low numbers of NK cells (83% vs 50% of patients) and T-cell clones in the blood (83% vs 30% of patients). RNA-sequencing analysis of an HVLPD skin lesion in a white patient compared with his normal skin showed increased expression of interferon-γ and chemokines that attract T cells and NK cells. Thus, white patients with HVLPD were less likely to have systemic disease with EBV and had a much better prognosis than nonwhite patients. This trial was registered at www.clinicaltrials.gov as #NCT00369421 and #NCT00032513.


Assuntos
Infecções por Vírus Epstein-Barr/patologia , Hidroa Vaciniforme/virologia , Transtornos Linfoproliferativos/patologia , Transtornos Linfoproliferativos/virologia , Criança , Pré-Escolar , Infecções por Vírus Epstein-Barr/etnologia , Infecções por Vírus Epstein-Barr/imunologia , Grupo com Ancestrais do Continente Europeu , Feminino , Humanos , Transtornos Linfoproliferativos/etnologia , Masculino
13.
Ann Diagn Pathol ; 40: 94-104, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31077877

RESUMO

There is a growing recognition that some primary cutaneous T cell lymphomas of the skin exhibit a follicular helper T cell phenotype best exemplified by primary cutaneous CD4+ small/medium sized pleomorphic T cell lymphoma. The follicular helper T cells is an evolutionary function in a common TH1 cell under the influence of other cell types most notably monocyte derived dendritic cells but also plasma cells. In addition, the skin defines a characteristic organ site of involvement for angioimmunoblastic T-cell lymphoma (AITL); the first recognized form of follicular helper T cell lymphoma. One of the hallmarks of the follicular helper T cell lymphomas a significant degree of post germinal center B cell hyperplasia. We encountered 7 cases of primary cutaneous follicular helper T cell and four cases of AITL, in which the biopsies contained a light chain restricted plasma cell infiltrate in the skin. There were no features that suggested an atypical or more aggressive clinical course in association with the identification of this light chain restricted plasmacytic infiltrates except one case of AITL in whom a diffuse large cell B cell lymphoma subsequently developed. There was no association with Epstein-Barr virus (EBV) infection light chain restricted plasma cell infiltrate in any of the eleven cases. The basis of these infiltrates is likely a reciprocal functional one reflecting the role of follicular helper T cells in the induction of B cell hyperplasia and the role of plasma cells as a countercheck balance controlling the extent of follicular helper T cell hyperplasia. B cell clonality, plasma cell atypia and blastic B cell transformation can occur without implying a malignant transformation.


Assuntos
Linfoma Folicular/patologia , Linfoma Cutâneo de Células T/patologia , Linfoma de Células T/patologia , Transtornos Linfoproliferativos/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Linfócitos B/patologia , Biópsia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Plasmócitos/patologia , Linfócitos T Auxiliares-Indutores/patologia
14.
Pediatr Blood Cancer ; 66(8): e27803, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31062898

RESUMO

Classic Hodgkin lymphoma post-transplant lymphoproliferative disorder (HL-PTLD) has been rarely reported in children, with limited data available to guide treatment decisions. We report a retrospective review of five children diagnosed with classic HL-PTLD following solid organ transplant between 2007 and 2013 at Stanford University. Patients were treated with Stanford V chemotherapy and involved field radiation therapy. With a median follow-up of 7.2 years (range, 4.7-10.5 years) since diagnosis, all patients remain in remission from HL-PTLD and free from graft failure. In this series, combined modality therapy with risk-adapted chemotherapy and radiation therapy was a successful strategy for the treatment of classic HL-PTLD.


Assuntos
Doença de Hodgkin/terapia , Transtornos Linfoproliferativos/terapia , Transplante de Órgãos/efeitos adversos , Complicações Pós-Operatórias/terapia , Criança , Pré-Escolar , Terapia Combinada , Feminino , Seguimentos , Doença de Hodgkin/etiologia , Doença de Hodgkin/patologia , Humanos , Lactente , Transtornos Linfoproliferativos/etiologia , Transtornos Linfoproliferativos/patologia , Masculino , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/patologia , Estudos Retrospectivos , Taxa de Sobrevida , Resultado do Tratamento
15.
Medicine (Baltimore) ; 98(19): e15365, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31083164

RESUMO

Rosai-Dorfman disease (RDD) with isolated central nervous system (CNS) involvement is an extremely rare disease. Most RDD of the CNS present as dural-based mass mimicking meningioma and other common lesions, which makes preoperative accurate diagnosis of great difficulty. We searched the pathology database in our hospital and 3 cases of RDD with isolated CNS involvement were finally included in our study. Radiological and clinical findings of these three cases were retrospectively analyzed. The lesions of 2 cases were dura-based against the cerebral convexity, presenting as a sheet-shaped thickened dura mater, another case was located just across the cerebral falx, the dural display in the center was intact. The 3 cases showed low signal intensity on T2-weighted image, obviously enhanced, significantly surrounding edema and finger-like protuberance but no invasion of the brain parenchyma or no sign of hyperplasia or sclerosis of the surrounding cranial bones. In conclusion, when we come across a disease that mimicking meningioma, especially when it manifests as the above radiological features, we should considered it might be a kind of proliferative disease of the meninges, such as RDD.


Assuntos
Doenças do Sistema Nervoso Central/diagnóstico por imagem , Doenças do Sistema Nervoso Central/patologia , Transtornos Linfoproliferativos/diagnóstico por imagem , Transtornos Linfoproliferativos/patologia , Meninges/diagnóstico por imagem , Meninges/patologia , Adulto , Encéfalo/diagnóstico por imagem , Doenças do Sistema Nervoso Central/cirurgia , Diagnóstico Diferencial , Feminino , Humanos , Transtornos Linfoproliferativos/cirurgia , Masculino , Meninges/cirurgia , Pessoa de Meia-Idade , Estudos Retrospectivos
16.
Front Biosci (Landmark Ed) ; 24: 1284-1315, 2019 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-31136980

RESUMO

Cellular therapies are a rapidly evolving approach to treat cancer in the light of their unique mechanism of action that potentially overcomes drug resistance and induces durable remissions. Modalities of adoptive cell therapy include gene-modified T cells expressing novel T cell receptors or chimeric antigen receptors (CAR) that modify the immune system to recognize tumor cells and carry out potent anti-tumor effector functions. CAR T cells have shown very promising clinical results and several trials are being conducted worldwide to establish their role in cancer treatment. Most successful results have been observed in lymphoproliferative disorders with the use of CD19-directed CAR T cells, which led to their commercial approval by FDA. In this review, we provide a comprehensive overview of the current role of CAR T cell therapies in hematological malignancies and solid tumors, their associated toxicities and potential future developments in the armamentarium for cancer treatment.


Assuntos
Imunoterapia Adotiva/métodos , Neoplasias/terapia , Receptores de Antígenos Quiméricos/imunologia , Linfócitos T/imunologia , Antígenos CD19/imunologia , Antígenos CD19/metabolismo , Neoplasias Hematológicas/imunologia , Neoplasias Hematológicas/patologia , Neoplasias Hematológicas/terapia , Humanos , Imunoterapia Adotiva/tendências , Transtornos Linfoproliferativos/imunologia , Transtornos Linfoproliferativos/patologia , Transtornos Linfoproliferativos/terapia , Neoplasias/imunologia , Neoplasias/patologia , Medicina de Precisão/métodos , Medicina de Precisão/tendências , Receptores de Antígenos Quiméricos/metabolismo , Linfócitos T/metabolismo , Resultado do Tratamento
17.
Pediatr Blood Cancer ; 66(8): e27798, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31099136

RESUMO

BACKGROUND: Systemic forms of EBV-associated T-cell lymphoproliferative disorders of childhood (S-EBV-T-LPD) comprise three major forms: EBV-positive hemophagocytic lymphohistiocytosis (EBV-HLH), systemic EBV-positive T-cell lymphoma (S-EBV-TCL), and systemic chronic active EBV infection (S-CAEBV). These disorders occur rarely in children in Western countries. Here, we described eight children of such entities. DESIGN: Eight cases (six clinical and two autopsy) with S-EBV-T-LPD of childhood were retrospectively identified from 1990 to 2015. Clinicopathologic parameters including histomorphology, immunophenotype, EBV studies, and T-cell receptor gene rearrangement studies were recorded. RESULTS: Patients include five females and three males of Hispanic, Asian, and Caucasian origins with an age range of 14 months to 9 years. Fever, hepatosplenomegaly, cytopenias, abnormal EBV serologies, and very high EBV viral loads were common findings. Histologic findings showed EBV+ T-cell infiltrates with variable degrees of architectural distortion and cytologic atypia ranging from no to mild cytologic atypia to overt lymphoma and tissue hemophagocytosis. All showed aberrant CD4+ or CD8+ T cells with dim to absent CD5, CD7, and CD3, and bright CD2 and CD45 by flow cytometry or loss of CD5 by immunohistochemistry. TCR gene rearrangement studies showed monoclonal rearrangements in all clinical cases (6/6). Outcomes were poor with treatment consisting of chemotherapy per the HLH-94 or HLH-2004 protocols with or without bone marrow transplant. CONCLUSION: In this large pediatric clinicopathologic study of S-EBV-T-LPD of childhood in the United States, EBV-HLH, S-EBV-TCL, and S-CAEBV show many overlapping features. Diagnosis is challenging, and overall outcome is poor using current HLH-directed therapies.


Assuntos
Infecções por Vírus Epstein-Barr/patologia , Herpesvirus Humano 4/isolamento & purificação , Linfoma de Células T/patologia , Transtornos Linfoproliferativos/patologia , Linfócitos T/patologia , Atenção Terciária à Saúde/estatística & dados numéricos , Medula Óssea/patologia , Criança , Pré-Escolar , Infecções por Vírus Epstein-Barr/complicações , Infecções por Vírus Epstein-Barr/virologia , Feminino , Seguimentos , Humanos , Lactente , Fígado/patologia , Linfonodos/patologia , Linfoma de Células T/classificação , Linfoma de Células T/etiologia , Transtornos Linfoproliferativos/classificação , Transtornos Linfoproliferativos/etiologia , Masculino , Prognóstico , Estudos Retrospectivos
18.
BMC Gastroenterol ; 19(1): 62, 2019 Apr 25.
Artigo em Inglês | MEDLINE | ID: mdl-31023238

RESUMO

BACKGROUND: Methotrexate-associated lymphoproliferative disorder (MTX-LPD) can present as a benign lymphoid proliferation or a malignant lymphoma in patients taking MTX. Almost 50% of MTX-LPD cases show spontaneous remission after withdrawal of MTX treatment. Studies have suggested that the hyper-immune state of rheumatoid arthritis, the immunosuppressive state associated with MTX, and the carcinogenicity of the Epstein-Barr virus might contribute to MTX-LPD development. Although most cases of MTX-LPD occur at extranodal sites, few cases of MTX-LPD affecting the stomach and duodenum have been reported. To our knowledge, no other study has reported on the endoscopic observations of dramatic withdrawal and appearance of multiple digestive tract lesions in a short period of time. Herein, we report the clinical course and imaging findings of our case, which may be useful for understanding the pathological condition of MTX-LPD. CASE PRESENTATION: We describe the case of a 70-year-old woman with MTX-LPD of the stomach and duodenum. Disease regression was temporarily achieved after cessation of MTX treatment; however, it subsequently recurred, and complete response was only achieved after six cycles of rituximab, cyclophosphamide, hydroxydaunorubicin, oncovin, and prednisolone (R-CHOP) chemotherapy. CONCLUSIONS: The first-choice therapy for patients taking MTX who develop suspected MTX-LPD should be the withdrawal of MTX treatment. Even after remission is achieved, patients should be kept under careful observation, and if the disease recurs, chemotherapy should be commenced promptly.


Assuntos
Antirreumáticos/efeitos adversos , Duodenopatias/induzido quimicamente , Transtornos Linfoproliferativos/induzido quimicamente , Metotrexato/efeitos adversos , Gastropatias/induzido quimicamente , Idoso , Anticorpos Monoclonais Murinos/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Artrite Reumatoide/tratamento farmacológico , Ciclofosfamida/uso terapêutico , Doxorrubicina/uso terapêutico , Duodenopatias/diagnóstico por imagem , Duodenopatias/tratamento farmacológico , Duodenopatias/patologia , Endoscopia do Sistema Digestório , Feminino , Humanos , Transtornos Linfoproliferativos/diagnóstico por imagem , Transtornos Linfoproliferativos/tratamento farmacológico , Transtornos Linfoproliferativos/patologia , Prednisona/uso terapêutico , Recidiva , Rituximab , Gastropatias/diagnóstico por imagem , Gastropatias/tratamento farmacológico , Gastropatias/patologia , Vincristina/uso terapêutico , Suspensão de Tratamento
20.
J Immunol ; 202(9): 2616-2627, 2019 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-30910863

RESUMO

Appropriate T cell responses are controlled by strict balance between activatory and inhibitory pathways downstream of TCR. Although mice or humans with impaired TCR signaling develop autoimmunity, the precise molecular mechanisms linking reduced TCR signaling to autoimmunity are not fully understood. Engagement of TCR activates Ca2+ signaling mainly through store-operated Ca2+ entry activated by stromal interaction molecule (Stim) 1 and Stim2. Despite defective T cell activation, mice deficient in both Stim1 and Stim2 in T cells (conditional double knockout [cDKO]) developed lymphoproliferative disorders and skin inflammation with a concomitant increase in serum IgG1 and IgE levels. In cDKO mice, follicular helper T (Tfh) cells were dramatically increased in number, and they produced IL-4 spontaneously. These inflammatory symptoms were abolished by the deletion of IL-4 in cDKO mice. Tfh development and inflammatory symptoms in cDKO mice were abrogated by further deletion of NFAT2 in T cells. These findings suggest that Tfh cells spontaneously developed in the absence of Ca2+ signaling and caused unregulated type 2 responses.


Assuntos
Diabetes Mellitus Experimental/imunologia , Diabetes Mellitus Tipo 2/imunologia , Doenças do Sistema Imunitário/imunologia , Molécula 1 de Interação Estromal/deficiência , Molécula 2 de Interação Estromal/deficiência , Linfócitos T Auxiliares-Indutores/imunologia , Animais , Sinalização do Cálcio/genética , Sinalização do Cálcio/imunologia , Diabetes Mellitus Experimental/genética , Diabetes Mellitus Experimental/patologia , Diabetes Mellitus Tipo 2/genética , Diabetes Mellitus Tipo 2/patologia , Doenças do Sistema Imunitário/genética , Doenças do Sistema Imunitário/patologia , Imunoglobulina E/genética , Imunoglobulina E/imunologia , Imunoglobulina G/genética , Imunoglobulina G/imunologia , Interleucina-4/genética , Interleucina-4/imunologia , Transtornos Linfoproliferativos/genética , Transtornos Linfoproliferativos/imunologia , Transtornos Linfoproliferativos/patologia , Camundongos , Camundongos Knockout , Fatores de Transcrição NFATC/genética , Fatores de Transcrição NFATC/imunologia , Dermatopatias/genética , Dermatopatias/imunologia , Dermatopatias/patologia , Linfócitos T Auxiliares-Indutores/patologia
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