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1.
Zhonghua Jie He He Hu Xi Za Zhi ; 47(6): 535-541, 2024 Jun 12.
Artigo em Chinês | MEDLINE | ID: mdl-38858203

RESUMO

Objective: To analyze of the clinical, imaging, and pathological features of pulmonary light chain deposition disease(PLCDD) secondary to Sjögren's syndrome(SS), and to improve the understanding of the disease. Methods: We retrospectively analyzed the clinical data of 23 PLCDD cases diagnosed by pathology in Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College from 2009 to 2023, a total of 11 cases of PLCDD secondary to SS(SS-PLCDD) were selected, the median age was 51 years old(range:36~74),10 female and 1 male, the clinical, imaging, and pathological features were summarized. Results: All 11 cases showed bilateral multiple pulmonary nodules on HRCT with 10 cases accompanied by cysts, 6 cases with vessels in the cystic wall or crossing the cysts. Microscopically, all 11 cases showed amorphous eosinophilic material with negative Congo red staining deposited in the lung, vascular involvement was common, with variable lymphocyte, plasma cell infiltration and multinucleated giant cell reaction, cysts formation was observed in 6 cases,1 case combined with pulmonary lymphoma. No extrapulmonary involvement in all 11 cases. Ten patients were treated with glucocorticoid and (or) immunosuppressants, 1 patient changed to bortezomib-dexamethasone chemotherapy after 1 year, with a mean follow-up of 50 months, 5 cases were stable on clinical and (or) HRCT findings, 2 cases showed remission on HRCT, 2 cases progressed on HRCT. Conclusions: SS-PLCDD affects predominantly middle-aged females with lesions confined to the lung; HRCT showed bilateral multiple nodules and thin-walled cysts. Pulmonary pathology presented as non-amyloid material with negative Congo red staining and interstitial changes associated with SS. The overall prognosis was good, but caution is advised regarding the underlying lymphoma.


Assuntos
Síndrome de Sjogren , Humanos , Síndrome de Sjogren/complicações , Feminino , Pessoa de Meia-Idade , Masculino , Estudos Retrospectivos , Adulto , Idoso , Pneumopatias/etiologia , Pneumopatias/patologia , Pulmão/patologia , Pulmão/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Cadeias Leves de Imunoglobulina/metabolismo
2.
Zhonghua Bing Li Xue Za Zhi ; 50(3): 222-228, 2021 Mar 08.
Artigo em Chinês | MEDLINE | ID: mdl-33677886

RESUMO

Objective: To analyze the clinicopathological and immunohistochemical characteristics of the hypersensitivity pneumonia (HP) cases that were diagnosed by excisional lung biopsy, to improve the diagnosis accuracy of HP. Methods: The data of 47 HP cases diagnosed by excisional lung biopsy during the last 20 years were collected in Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, from January 2000 to June 2020. The clinicopathological features and the immunohistochemical profiles of CD3, CD4, CD8 and CD20 were analyzed using light microscopy and immunohistochemical stains. Results: The age of the patients ranged from 15 to 68 years, while the ratio of male to female was 22∶25. The main symptoms were shortness of breath and cough, and 32 cases had a history of allergen exposure. Most of the patients had restrictive impaired ventilatory function, and impaired diffusion function. The proportion of lymphocytes in bronchoalveolar lavage fluid (BALF) increased in 85.3%(29/34) of the patients, and the CD4+/CD8+ T cell ratio was not higher than 0.8 in 31.3%(10/32) of the patients. Microscopically, 59.6%(28/47) of the cases showed nonnecrotizing granuloma/giant cells in the stroma, and 40.4% (19/47) of the cases had only giant cells. Ninety-three-point six percent of the cases(44/47) had peribronchiolar metaplasia, while 70.2%(33/47) had focal cell-rich inflammation. Forty-four cases (93.6%, 44/47) had fibrosis, which was manifested as usual interstitial pneumonia-like fibrosis (47.7%, 21 cases), nonspecific interstitial pneumonia-like fibrosis (29.5%, 13 cases), purely peribronchiolar fibrosis (6.8%, 3 cases) and mixed fibrosis (15.9%, 7 cases). Immunohistochemistry showed that CD4+ T cells were fewer than CD8+ T cells in 24 cases (55.8%, 24/43) of HP. Thirty-two HP patients had follow-up data. Among them, 7 patients had improved symptoms, 12 patients were stable, and 13 patients had worsening symptoms. Conclusions: The most common type of HP diagnosed by excisional lung biopsy is chronic HP. The clinical characteristics are long course of disease, restricted ventilation disorder and impaired diffusion function with the increase of lymphocyte proportion in BALF. Nearly 1/3 of the patients have no known history of allergen contact. The degree of fibrosis in HP patients diagnosed by excisional lung biopsy is severe, and the histologic types of fibrosis varies, but the UIP-like and/or NSIP-like fibrosis is most common. The CD4+/CD8+ T cell ratio in lung tissues is less than 1 in more than half of the cases. Pulmonary fibrosis continued to increase in some of the patients with chronic HP, even after treatment.


Assuntos
Alveolite Alérgica Extrínseca , Fibrose Pulmonar , Adolescente , Adulto , Idoso , Alveolite Alérgica Extrínseca/diagnóstico , Biópsia , Linfócitos T CD8-Positivos , Feminino , Humanos , Pulmão/cirurgia , Masculino , Pessoa de Meia-Idade , Adulto Jovem
3.
Zhonghua Bing Li Xue Za Zhi ; 49(6): 583-587, 2020 Jun 08.
Artigo em Chinês | MEDLINE | ID: mdl-32486535

RESUMO

Objective: To compare the differences in the detection rates of cytomegalovirus (CMV) infection between the left and right colons for guiding endoscopic biopsy-sites. Methods: The cases of colonic CMV infection confirmed by histology at the Peking Union Medical College Hospital from July 2015 to July 2018 were collected and analyzed. Splenic curvature was used as the dividing point between the left and right colons. The CMV inclusions were identified histologically, and the CMV infected cells were detected using immunohistochemistry (EnVision method). The numbers of infected cells in the left and right colons were compared by histological and immunohistochemical results. A total of 731 patients were identified. Ten of the 731 patients had histological specimens of both left and right colons and were confirmed with CMV EnVision immunohistochemical study. There were 25 biopsy or resection specimens. Seven patients were male and 3 were female, and their ages ranged from 29 to 66 years, with a median age of 55 years. All of the 10 patients were also diagnosed with ulcerative colitis. Results: The number of the cells infected with CMV in the left colon was 115 (1-41), while that in the right colon was 76 (0-51). In 8/10 cases, the number of CMV infected cells in the left colon was more than that in the right colon. Conclusions: The study on the biopsies and resection specimens suggests that CMV infection is mostly present in the left colon. The left colon thus might be an important endoscopic biopsy-site for the cases with suspicion of CMV infection.


Assuntos
Colite Ulcerativa , Infecções por Citomegalovirus , Adulto , Idoso , Biópsia , Citomegalovirus , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
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