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1.
BMJ Case Rep ; 16(12)2023 Dec 16.
Artigo em Inglês | MEDLINE | ID: mdl-38103907

RESUMO

Lymphomatoid granulomatosis (LYG) is a rare B cell lymphoproliferative disorder associated with Epstein-Barr virus infection. LYG diagnosis is often difficult because of non-specific and varied radiological and pathological findings. The lung is the most common organ of LYG occurrence, but extrapulmonary lesions involving the central nervous system, skin, kidneys and liver are observed. A surgical biopsy is often inevitable for LYG diagnosis.We encountered a man in his 50s who presented with progressive dyspnoea. Extrapulmonary lesions were not observed. Although he developed respiratory failure within a short period, a low dose of corticosteroid relieved his symptoms. Video-assisted thoracoscopic lung biopsy revealed grade 1 LYG. The patient was successfully treated with chemotherapy, including rituximab. Only a few cases of LYG with progressive respiratory failure are reported, and most have been diagnosed via autopsy. Our case highlights the importance of performing a surgical lung biopsy at the appropriate time to diagnose LYG.


Assuntos
Infecções por Vírus Epstein-Barr , Granulomatose Linfomatoide , Síndrome do Desconforto Respiratório , Insuficiência Respiratória , Masculino , Humanos , Granulomatose Linfomatoide/complicações , Granulomatose Linfomatoide/diagnóstico , Granulomatose Linfomatoide/tratamento farmacológico , Infecções por Vírus Epstein-Barr/complicações , Herpesvirus Humano 4 , Pulmão/diagnóstico por imagem , Pulmão/patologia , Síndrome do Desconforto Respiratório/complicações , Insuficiência Respiratória/complicações
2.
J Pediatr Hematol Oncol ; 45(7): e927-e930, 2023 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-37526440

RESUMO

BACKGROUND: Lymphomatoid granulomatosis (LYG) is a rare pediatric disorder driven by the Epstein-Barr virus and is considered as a part of the lymphoma spectrum. It is mostly associated with immune deficiency and patients on immunosuppressive therapy, especially with acute leukemia. It can present as a multisystemic disease, diagnosed on biopsy as atypical lymphocytes with an angiocentric pattern against a background composed of histiocytes, neutrophils, and extensive T-cell infiltration. OBSERVATION: We report 3 cases of children with Lymphomatoid granulomatosis, one with Langerhans cell histiocytosis. CONCLUSION: Combination chemotherapy was used for the treatment of Lymphomatoid granulomatosis; however, the prognosis is guarded. One of 3 patients is alive and in remission on the last follow-up visit at 15 months.


Assuntos
Infecções por Vírus Epstein-Barr , Granulomatose Linfomatoide , Humanos , Criança , Granulomatose Linfomatoide/diagnóstico , Granulomatose Linfomatoide/tratamento farmacológico , Granulomatose Linfomatoide/patologia , Herpesvirus Humano 4 , Infecções por Vírus Epstein-Barr/complicações , Infecções por Vírus Epstein-Barr/patologia , Paquistão , Linfócitos T/patologia
3.
BMC Neurol ; 23(1): 208, 2023 May 29.
Artigo em Inglês | MEDLINE | ID: mdl-37248444

RESUMO

BACKGROUND: Lymphomatoid granulomatosis (LyG) is a rare extralymphatic lymphoproliferative disease characterized by lymphocytic invasion into vascular walls and damage to blood vessels. The lungs are affected in 90% of LyG cases, followed by the skin, central nervous system (CNS), kidneys and liver. CASE PRESENTATION: Here we report a case of a young woman with LyG, with CNS involvement as the initial clinical manifestation. Computer tomography (CT) scans showed multiple nodular, patchy and flocculent high-density shadows in both lungs without mediastinal lymph node enlargement. Magnetic resonance imaging (MRI) scans showed multiple abnormal signal intensities in the right cerebellar hemisphere, frontal, parietal and temporal lobes, and dorsal brainstem, which became patchy and annular after enhancement. The post-operative pathological analysis of lesion samples confirmed the diagnosis of grade II LyG. CONCLUSIONS: LyG should be concerned in young adults showing multiple radiological brain and lung lesions. Resection and postoperative medication of steroid hormones and IFN-α may be effective in the treatment of LyG.


Assuntos
Neoplasias Encefálicas , Granulomatose Linfomatoide , Feminino , Adulto Jovem , Humanos , Granulomatose Linfomatoide/diagnóstico por imagem , Sistema Nervoso Central/patologia , Pulmão/patologia , Neoplasias Encefálicas/patologia , Encéfalo/patologia
4.
BMJ Case Rep ; 16(5)2023 May 09.
Artigo em Inglês | MEDLINE | ID: mdl-37160375

RESUMO

A man in his 60s presented with intermittent constitutional symptoms along with waxing and waning chest radiographic abnormalities, eventually leading to a diagnosis of lymphomatoid granulomatosis (LYG). LYG is a rare, progressive Epstein-Barr virus (EBV)-driven lymphoproliferative disease associated with immune dysregulation most commonly involving the lungs. The diagnosis requires tissue biopsy; thus, the decision to pursue tissue sampling with histopathology examination in a timely manner is essential. Currently, there are no established guidelines regarding the treatment of LYG, which varies from cessation of immunosuppressants to immunochemotherapy and usually requires multidisciplinary team discussion.


Assuntos
Infecções por Vírus Epstein-Barr , Granulomatose Linfomatoide , Masculino , Humanos , Fator de Necrose Tumoral alfa , Infecções por Vírus Epstein-Barr/complicações , Infecções por Vírus Epstein-Barr/tratamento farmacológico , Granulomatose Linfomatoide/induzido quimicamente , Granulomatose Linfomatoide/diagnóstico , Granulomatose Linfomatoide/tratamento farmacológico , Herpesvirus Humano 4 , Fatores Imunológicos
6.
Lancet Haematol ; 10(5): e346-e358, 2023 May.
Artigo em Inglês | MEDLINE | ID: mdl-37011643

RESUMO

BACKGROUND: Lymphomatoid granulomatosis is a rare Epstein-Barr virus-associated B-cell lymphoproliferative disorder with a median overall survival of less than 2 years. In this study, we hypothesised that low-grade lymphomatoid granulomatosis is immune-dependent and high-grade lymphomatoid granulomatosis is immune-independent. On the basis of this hypothesis, we investigated the activity and safety of new treatment with immunotherapy in patients with low-grade disease and standard chemotherapy in patients with high-grade disease. METHODS: In this open-label, single-centre, phase 2 trial, we enrolled patients aged 12 years or older with untreated, or relapsed or refractory lymphomatoid granulomatosis at the National Cancer Institute (National Institutes of Health, Bethesda, MD, USA). Patients with low-grade disease received dose-escalated interferon alfa-2b, starting at 7·5 million international units subcutaneously three times per week for up to 1 year past best response, and patients with high-grade disease received six cycles every 3 weeks of intravenous, dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, and rituximab (DA-EPOCH-R). Starting doses were 50 mg/m2 per day as a continuous intravenous infusion from day 1 to day 4 (96 h) for etoposide; 60 mg/m2 twice daily by mouth from day 1 to day 5 for prednisone; 0·4 mg/m2 per day as a continuous intravenous infusion from day 1 to day 4 (96 h) for vincristine; 750 mg/m2 intravenous on day 5 for cyclophosphamide; 10 mg/m2 per day as a continuous intravenous infusion from day 1 to day 4 (96 h) for doxorubicin; and 375 mg/m2 intravenous on day 1 for rituximab. The doses of doxorubicin, etoposide, and cyclophosphamide were adjusted up or down on the basis of neutrophil and platelet nadirs. Patients with residual or progressive disease after initial therapy crossed over to alternative therapy. The primary endpoint was the proportion of patients who had an overall response and the 5-year progression-free survival after initial or cross-over treatment. Analysis of response included all participants who underwent restaging imaging; safety analysis included all patients who received any dose of study drugs. The trial is open for enrolment and is registered at ClinicalTrials.gov, NCT00001379. FINDINGS: 67 patients were enrolled between Jan 10, 1991, and Sept 5, 2019 (42 [63%] were male). 45 patients received initial treatment with interferon alfa-2b (16 of whom crossed over to DA-EPOCH-R) and 18 received initial treatment with DA-EPOCH-R (eight of whom crossed over to interferon alfa-2b); four underwent surveillance only. After initial treatment with interferon alfa-2b, the overall response was 64% (28 of 44 evaluable patients) with 61% (27 of 44) having a complete response, whereas, after cross-over treatment with interferon alfa-2b, the overall response was 63% (five of eight evaluable patients) with 50% (four of eight) having a complete response. After initial treatment with DA-EPOCH-R, the overall response was 76% (13 of 17 evaluable patients) with 47% (eight of 17) having a complete response, whereas, after cross-over treatment with DA-EPOCH-R, the overall response was 67% (ten of 15 evaluable patients) with 47% (seven of 15) having a complete response. 5-year progression-free survival was 48·5% (95% CI 33·2-62·1) after initial treatment with interferon alfa-2b, 50·0% (15·2-77·5) after cross-over treatment with interferon alfa-2b, 25·4% (8·2-47·2) after initial treatment with DA-EPOCH-R, and 62·5% (34·9-81·1) after cross-over treatment with DA-EPOCH-R. The most common grade 3 or worse adverse events in patients treated with interferon alfa-2b included neutropenia (27 [53%] of 51 patients), lymphopenia (24 [47%]), and leukopenia (24 [47%]). The four most common grade 3 or worse adverse events in patients treated with DA-EPOCH-R included neutropenia (29 [88%] of 33 patients), leukopenia (28 [85%]), infection (18 [55%]), and lymphopenia (17 [52%]). Serious adverse events occurred in 13 (25%) of 51 patients receiving treatment with interferon alfa-2b and 21 (64%) of 33 patients receiving DA-EPOCH-R, with five treatment-related deaths: one thromboembolic, one infection, and one haemophagocytic syndrome with interferon alfa-2b, and one infection and one haemophagocytic syndrome with DA-EPOCH-R. INTERPRETATION: Interferon alfa-2b is efficacious for treating low-grade lymphomatoid granulomatosis and hence reducing progression to high-grade disease, whereas patients with high-grade lymphomatoid granulomatosis showed expected responses to chemotherapy. Uncontrolled immune regulation of Epstein-Barr virus is hypothesised to result in the emergence of low-grade disease after chemotherapy, for which treatment with interferon alfa-2b is efficacious. FUNDING: Intramural Research Programs of the National Cancer Institute and National Institute of Allergy and Infectious Diseases, National Institutes of Health.


Assuntos
Infecções por Vírus Epstein-Barr , Linfo-Histiocitose Hemofagocítica , Linfoma Difuso de Grandes Células B , Linfoma não Hodgkin , Granulomatose Linfomatoide , Linfopenia , Neutropenia , Humanos , Masculino , Feminino , Vincristina/efeitos adversos , Prednisona/uso terapêutico , Etoposídeo/uso terapêutico , Rituximab/efeitos adversos , Interferon alfa-2/uso terapêutico , Infecções por Vírus Epstein-Barr/induzido quimicamente , Infecções por Vírus Epstein-Barr/tratamento farmacológico , Linfo-Histiocitose Hemofagocítica/tratamento farmacológico , Granulomatose Linfomatoide/tratamento farmacológico , Granulomatose Linfomatoide/induzido quimicamente , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Herpesvirus Humano 4 , Linfoma não Hodgkin/tratamento farmacológico , Ciclofosfamida/efeitos adversos , Doxorrubicina/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Neutropenia/etiologia , Linfopenia/induzido quimicamente , Linfopenia/tratamento farmacológico
7.
Dermatol Online J ; 29(1)2023 Feb 15.
Artigo em Inglês | MEDLINE | ID: mdl-37040909

RESUMO

Methotrexate (MTX) is a first-line systemic medication used to treat rheumatoid arthritis because of its immunomodulatory effects. However, MTX has also been linked to the development of lymphoproliferative disorders (LPD) in patients with rheumatoid arthritis. We describe a patient with long-standing rheumatoid arthritis treated with MTX who developed cutaneous Epstein-Barr virus (EBV)-positive B cell lymphoproliferative disease resembling grade III lymphomatoid granulomatosis localized to the right leg. The lymphomatoid process resolved with withdrawal of the MTX. The pathogenesis of iatrogenic lymphoproliferative disorder was most likely triggered by the rheumatoid inflammation and the immunosuppressing effects of MTX, which led to EBV reactivation. We recommend a trial of MTX discontinuation prior to considering chemotherapy in patients with rheumatoid arthritis treated with MTX who develop EBV-positive B cell lymphoproliferative disease resembling a high grade B-cell lymphoma.


Assuntos
Artrite Reumatoide , Infecções por Vírus Epstein-Barr , Granulomatose Linfomatoide , Transtornos Linfoproliferativos , Humanos , Artrite Reumatoide/tratamento farmacológico , Infecções por Vírus Epstein-Barr/induzido quimicamente , Herpesvirus Humano 4 , Granulomatose Linfomatoide/induzido quimicamente , Granulomatose Linfomatoide/tratamento farmacológico , Metotrexato/uso terapêutico , Antígeno Ki-1/imunologia , Transtornos Linfoproliferativos/complicações , Transtornos Linfoproliferativos/imunologia , Linfócitos B/imunologia
9.
Ear Nose Throat J ; 102(4): NP154-NP156, 2023 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33634720

RESUMO

Epstein-Barr virus (EBV) associated lymphoproliferative disorders includes a diverse group of diagnoses, encompassing both B-cell and T-cell lineages. With EBV mucocutaneous ulcers becoming a World health Organization diagnosis in 2018, introduction of the disease entity will be beneficial to the practicing otolaryngologist. We are reporting a case of a 69-year-old male with history of rheumatoid arthritis on methotrexate, recently undergoing dental extractions, who then developed multiple oral ulcerations and bony erosions of his palate and alveolar ridge. Associated symptoms included a large 3.0 cm neck mass, splenomegaly, and pulmonary nodules. Histopathology showed EBV+ lymphomatoid granulomatosis. Upon removal of immunosuppressive agent, patient's symptoms improved with resolution of oral lesions, as well as systemic symptoms.


Assuntos
Infecções por Vírus Epstein-Barr , Granulomatose Linfomatoide , Masculino , Humanos , Idoso , Granulomatose Linfomatoide/complicações , Granulomatose Linfomatoide/patologia , Herpesvirus Humano 4 , Infecções por Vírus Epstein-Barr/complicações , Linfócitos B/patologia , Extração Dentária
10.
Intern Med ; 62(13): 1977-1982, 2023 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-36418093

RESUMO

Lymphomatoid granulomatosis (LYG) is a rare lymphoproliferative disorder (LPD). The optimal management strategy of methotrexate (MTX) related-LPD with central nervous system (CNS) involvement and histological features of LYG remains unclear. We herein report a case of grade 2-3 LYG in a rheumatoid arthritis patient, in which an intracranial mass accompanied by hemorrhaging and pulmonary and skin lesions developed. The patient received successful rituximab monotherapy. The tumor cells in the skin and brain showed monoclonal and oligoclonal proliferation, respectively. Our case suggests that rituximab monotherapy may be effective against MTX-LPD with CNS involvement, especially in cases with LYG histology.


Assuntos
Artrite Reumatoide , Granulomatose Linfomatoide , Humanos , Metotrexato/efeitos adversos , Granulomatose Linfomatoide/induzido quimicamente , Granulomatose Linfomatoide/tratamento farmacológico , Granulomatose Linfomatoide/patologia , Rituximab/efeitos adversos , Artrite Reumatoide/tratamento farmacológico , Encéfalo/patologia
12.
J Cancer Res Clin Oncol ; 149(9): 5729-5732, 2023 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-36566338

RESUMO

Primary lymphomatoid granulomatosis of the CNS (CNS-LG) is a rare lymphoid neoplasia associated Epstein-Barr Virus (EBV) and often accompanied by immunodeficiencies. No treatment standards have been defined yet. However, due to often devastating neurologic sequelae and based on similarities to diffuse large B-cell lymphoma, curative treatment requires intensive therapy protocols resembling protocols applied in CNS lymphoma. Here, the clinical courses and treatments of four primary CNS-LG patients in analogy to aggressive CNS-lymphomas including methotrexate, thiotepa, cytarabine, carmustine, and rituximab are presented. This is the first report on high-dose chemotherapy with CNS-directed drugs and autologous blood stem cell transplantation in primary CNS-LG.


Assuntos
Antineoplásicos , Protocolos de Quimioterapia Combinada Antineoplásica , Viroses do Sistema Nervoso Central , Infecções por Vírus Epstein-Barr , Granulomatose Linfomatoide , Transplante de Células-Tronco de Sangue Periférico , Granulomatose Linfomatoide/tratamento farmacológico , Granulomatose Linfomatoide/cirurgia , Metotrexato/uso terapêutico , Tiotepa/uso terapêutico , Citarabina/uso terapêutico , Carmustina/uso terapêutico , Rituximab/uso terapêutico , Viroses do Sistema Nervoso Central/tratamento farmacológico , Viroses do Sistema Nervoso Central/cirurgia , Infecções por Vírus Epstein-Barr/tratamento farmacológico , Infecções por Vírus Epstein-Barr/cirurgia , Humanos , Antineoplásicos/uso terapêutico , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Transplante Autólogo , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Resultado do Tratamento
13.
Front Immunol ; 13: 1001055, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36389673

RESUMO

A high prevalence of Epstein-Barr virus (EBV) infection in patients with inflammatory bowel disease (IBD) has been reported in many case reports and studies; thus, the association between EBV and IBD has gained increasing attention. Patients with IBD are at an increased risk of opportunistic EBV infection owing to the common use of immunomodulators. EBV infection in IBD patients can cause various complications, including superimposed viral colitis, which is associated with chronicity, exacerbation, and poor prognosis of refractory IBD, and can induce progression to lymphoproliferative disorders, such as EBV-positive mucocutaneous ulcer (EBVMCU), lymphomatoid granulomatosis (LYG), hemophagocytic lymphohistiocytosis (HLH) and diffuse large B-cell lymphoma (DLBCL). It has been suggested to screen for EBV before initiating immunosuppressive therapy and monitor the status of EBV infection in patients with IBD, especially those who are EBV-seronegative and have a risk of primary EBV infection. Clinicians should also be careful of misdiagnosing IBD and EBV-associated lymphoproliferative diseases due to similarities in both clinical symptoms and endoscopic manifestations. Withdrawal of immunosuppressants has been shown to be an effective strategy to achieve remission of disease at the time of EBV diagnosis, but antiviral therapy remains controversial. The present review aims to describe the characteristics of the complications caused by EBV infection and generalize the recent research progress on and challenges caused by EBV infection in IBD patients. The literature for writing this review was collected from 'PubMed' research engine. The keywords 'inflammatory bowel disease and Epstein-Barr virus' or 'ulcerative colitis and Epstein-Barr virus' or 'Crohn's disease and Epstein-Barr virus' were used to collect the literature and relevant papers were collected to help writing this review.


Assuntos
Colite Ulcerativa , Infecções por Vírus Epstein-Barr , Doenças Inflamatórias Intestinais , Granulomatose Linfomatoide , Humanos , Herpesvirus Humano 4 , Doenças Inflamatórias Intestinais/complicações , Doenças Inflamatórias Intestinais/tratamento farmacológico , Doenças Inflamatórias Intestinais/diagnóstico , Colite Ulcerativa/terapia
14.
Clin Nucl Med ; 47(12): e749-e751, 2022 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-36342805

RESUMO

ABSTRACT: A 52-year-old woman complained of upper respiratory symptoms and subsequently developed Wallenberg syndrome. Chest CT and brain MRI revealed multiple nodular lesions in the lungs and brain. She was pathologically diagnosed with low-grade lymphomatoid granulomatosis by lung biopsy. Brain PET examinations using 11C-methionine, 18F-FDG, and 18F-THK5351 were performed. Uptake of 11C-methionine and 18F-FDG was slightly increased in some lesions, likely reflecting the degree of inflammatory cell infiltration. 18F-THK5351 uptake was significantly increased in all lesions, likely reflecting the degree of reactive astrogliosis. This case illustrates the utility of PET studies for diagnosing lymphomatoid granulomatosis and provides insight into its pathophysiology.


Assuntos
Fluordesoxiglucose F18 , Granulomatose Linfomatoide , Feminino , Humanos , Pessoa de Meia-Idade , Radioisótopos de Carbono , Granulomatose Linfomatoide/diagnóstico por imagem , Granulomatose Linfomatoide/patologia , Compostos Radiofarmacêuticos , Tomografia por Emissão de Pósitrons/métodos , Metionina , Encéfalo/diagnóstico por imagem , Encéfalo/patologia
16.
Am J Case Rep ; 23: e936862, 2022 Aug 03.
Artigo em Inglês | MEDLINE | ID: mdl-35918872

RESUMO

BACKGROUND Lymphomatoid granulomatosis (LyG) is a rare lymphoproliferative disorder associated with Epstein-Barr virus (EBV) in which there is an infection of B cells and numerous reactive T cells. The lymphoproliferative disorder progresses to organ infiltration and resultant dysfunction of affected organs. Histologically, it is characterized by a triad of polymorphic lymphoid infiltrate, angiitis, and granulomatosis. The lungs are the most commonly involved sites for lymphomatoid granulomatosis, but other sites that can be involved include the liver, skin, and central nervous system. The signs and symptoms of LyG can vary, and can produce generalized symptoms such as cough, shortness of breath, and chest tightness, but can vary depending on the location of LyG. CASE REPORT We report a case of a 60-year-old man who presented with altered mental status. Cross-sectional imaging of the brain was negative for any acute intracranial process, but a fine-needle biopsy of a retroperitoneal lymph node revealed nodular polymorphous mononuclear infiltrates containing atypical large EBV-positive B cells with positive EBER and CD30, consistent with lymphomatoid granulomatosis. The patient was started on a regimen of brentuximab/bendamustine, and instructed to follow up with Oncology on an outpatient basis. CONCLUSIONS Treatment options for lymphomatoid granulomatosis are based on the disease grading. Lymphomatoid granulomatosis can be classified by using a grading system determined by the number of EBV-positive large B cell malignant cells, along with necrosis. The most effective treatment for lymphomatoid granulomatosis is unknown, but at this time treatment protocols are based on the grade of the disease. The clinical and histological features of lymphomatoid granulomatosis are discussed in this case report.


Assuntos
Infecções por Vírus Epstein-Barr , Granulomatose Linfomatoide , Infecções por Vírus Epstein-Barr/complicações , Herpesvirus Humano 4 , Humanos , Pulmão/patologia , Linfonodos/patologia , Granulomatose Linfomatoide/complicações , Granulomatose Linfomatoide/diagnóstico , Granulomatose Linfomatoide/patologia , Masculino , Pessoa de Meia-Idade
18.
Chest ; 161(1): e35-e41, 2022 01.
Artigo em Inglês | MEDLINE | ID: mdl-35000715

RESUMO

CASE PRESENTATION: A 71-year-old man with history of gastroesophageal reflux disease, chronic sinusitis, arthritis, hypothyroidism, and anemia of chronic disease initially sought treatment with a recurrent left pleural effusion along with other abnormal lung findings on chest CT scan. Before his referral, he was being managed for 3 years at his local hospital for waxing and waning fevers, fatigue, productive cough, chills, and night sweats. He did not report any hemoptysis or chest pain, but reported weight loss of 13 kgs in 15 months. During those 3 years, he was treated with multiple courses of antibiotics and steroids with temporary relief of symptoms. At that time, his chronic sinusitis was suspected to be the cause of his symptoms and he underwent balloon sinuplasty. He was receiving daily sublingual immunotherapy for inhaled respiratory allergens for the previous year after showing positive test results for 17 inhaled allergens. The patient had no other known immunologic workup before our evaluation.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Pulmão/diagnóstico por imagem , Granulomatose Linfomatoide/diagnóstico , Idoso , Broncoscopia , Ciclofosfamida/uso terapêutico , Doxorrubicina/uso terapêutico , Empiema/fisiopatologia , Infecções por Vírus Epstein-Barr , Febre/fisiopatologia , Humanos , Leucocitose/fisiopatologia , Pulmão/patologia , Granulomatose Linfomatoide/tratamento farmacológico , Granulomatose Linfomatoide/fisiopatologia , Granulomatose Linfomatoide/virologia , Masculino , Prednisona/uso terapêutico , Rituximab/uso terapêutico , Tomografia Computadorizada por Raios X , Vincristina/uso terapêutico
19.
J Neuroimmunol ; 361: 577748, 2021 12 15.
Artigo em Inglês | MEDLINE | ID: mdl-34653948

RESUMO

We report two patients with meningoencephalomyelitis without evidence of extra central nervous system (CNS) involvement. Brain MRI showed linear perivascular radial gadolinium enhancement patterns and spinal cord MRI showed longitudinal extensive T2-hyperintensity lesions. Pathological findings from brain biopsies were angiocentric T-cell predominant lymphoid infiltrates that lacked Epstein-Barr virus-positive atypical B cells. The patients were initially suspected to have isolated CNS-lymphomatoid granulomatosis (LYG). Thereafter, glial fibrillary acidic protein (GFAP)-immunoglobulin G were detected in their cerebrospinal fluid. This finding suggested autoimmune GFAP astrocytopathy. We speculate there is a link between isolated CNS-LYG and autoimmune GFAP astrocytopathy.


Assuntos
Astrócitos/patologia , Doenças Autoimunes do Sistema Nervoso/diagnóstico , Proteína Glial Fibrilar Ácida/imunologia , Granulomatose Linfomatoide/diagnóstico , Corticosteroides/uso terapêutico , Idoso , Especificidade de Anticorpos , Astrócitos/imunologia , Autoanticorpos/líquido cefalorraquidiano , Doenças Autoimunes do Sistema Nervoso/líquido cefalorraquidiano , Doenças Autoimunes do Sistema Nervoso/imunologia , Doenças Autoimunes do Sistema Nervoso/patologia , Encéfalo/diagnóstico por imagem , Encéfalo/imunologia , Encéfalo/patologia , Diagnóstico Diferencial , Humanos , Imunoglobulina G/líquido cefalorraquidiano , Imunoglobulinas Intravenosas/uso terapêutico , Imageamento por Ressonância Magnética , Masculino , Meningoencefalite/etiologia , Pessoa de Meia-Idade , Mielite/etiologia , Neuroimagem , Estudos Retrospectivos , Medula Espinal/diagnóstico por imagem , Medula Espinal/imunologia , Medula Espinal/patologia
20.
Rinsho Shinkeigaku ; 61(9): 618-623, 2021 Sep 28.
Artigo em Japonês | MEDLINE | ID: mdl-34433742

RESUMO

A 71-year-old man was hospitalized because of low back pain and weakness in both lower limbs. He presented with fever and stiff neck, and his cerebrospinal fluid sample contained blood. MRI revealed intramedullary and epidural hemorrhages in the spinal cord. Microhemorrhages occurred frequently in the central nervous system over a short period. A brain biopsy was performed. The diagnosis was primary lymphomatoid granulomatosis (LYG) of the central nervous system (grade 2). As a result of lymphocytic infiltration to the vascular walls in LYG, hemorrhages occurred in multiple sites in the central nervous system. The biopsy of samples from the sites of microhemorrhages proved useful for diagnosis even in the absence of mass lesions.


Assuntos
Granulomatose Linfomatoide , Idoso , Encéfalo/diagnóstico por imagem , Sistema Nervoso Central , Humanos , Granulomatose Linfomatoide/diagnóstico por imagem , Imageamento por Ressonância Magnética , Masculino , Medula Espinal
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