RESUMO
Chronic granulomatous disease (CGD) should be considered as a differential diagnosis in children and adolescents with frequent infections, especially when caused by certain specific pathogens.This case report describes a 64-year-old female with multiple recurrent and complicated bronchopulmonary infections, caused by common, but also rare pathogens, autoimmune phenomena, malignancies and recurrent organizing pneumonia (OP) with granulomas. Finally, the patient was diagnosed with p47phox-deficient chronic granulomatous disease (CGD).Individuals with a primary immunodeficiency may survive multiple complications and may be diagnosed at an advanced age especially if the affected structure shows residual activity. When confronted with patients with recurrent bronchopulmonary infections, especially with certain specific rare pathogens, in combination with organizing pulmonary granulomas as well as autoimmune phenomena, CGD should be considered even in elderly patients. Delayed diagnosis significantly increases mortality and morbidity in such cases.
Assuntos
Doença Granulomatosa Crônica/diagnóstico , Pneumonia/diagnóstico , Diagnóstico Diferencial , Feminino , Doença Granulomatosa Crônica/complicações , Humanos , Infecções , Pessoa de Meia-Idade , Pneumonia/etiologiaRESUMO
The aim of the present study was to investigate a case of hypersensitivity pneumonitis associated with the use of temozolomide in the treatment of gliosarcoma. A 54-yr-old female developed dyspnoea, cough and hypoxia after surgical resection for gliosarcoma and adjuvant radio- and chemotherapy with temozolomide. A high-resolution computed tomography scan of the thorax showed a bilateral ground-glass pattern. Bronchoscopy with bronchoalveolar lavage and lung biopsies was performed. Bronchoalveolar lavage demonstrated significant lymphocytic alveolitis and transbronchial lung biopsies revealed lymphocytic infiltration with foamy macrophages, consistent with hypersensitivity pneumonitis. There was no evidence of other causes, including infections. After withdrawing temozolomide and initiating prednisolone therapy, the patient had no further pulmonary symptoms. To the present authors' knowledge, this is the first definitively described case of temozolomide-associated hypersensitivity pneumonitis.
Assuntos
Alveolite Alérgica Extrínseca/induzido quimicamente , Antineoplásicos Alquilantes/efeitos adversos , Dacarbazina/análogos & derivados , Alveolite Alérgica Extrínseca/diagnóstico por imagem , Alveolite Alérgica Extrínseca/tratamento farmacológico , Biópsia , Broncoscopia , Dacarbazina/efeitos adversos , Feminino , Glucocorticoides/uso terapêutico , Humanos , Pessoa de Meia-Idade , Prednisolona/uso terapêutico , Temozolomida , Tomografia Computadorizada por Raios XAssuntos
Carcinoma de Células Renais/secundário , Perfilação da Expressão Gênica , Neoplasias Renais/genética , Neoplasias Pulmonares/secundário , Carcinoma de Células Renais/genética , Carcinoma de Células Renais/patologia , Carcinoma de Células Renais/cirurgia , DNA de Neoplasias/genética , Regulação Neoplásica da Expressão Gênica/fisiologia , Humanos , Neoplasias Renais/patologia , Neoplasias Renais/cirurgia , Terapia a Laser , Pulmão/patologia , Pulmão/cirurgia , Neoplasias Pulmonares/genética , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/cirurgia , Análise de Sequência com Séries de Oligonucleotídeos , Polimorfismo de Nucleotídeo Único/genética , PrognósticoRESUMO
Malignant fibrous histiocytoma (MFH), arising in combination with a sacral chordoma in a 70-year-old men, is described. Intermediate spindle-shaped cells demonstrating keratin positivity, showed a gradual transition between the areas of conventional chordoma, and the spindle cell areas, lending credence to the theory of a multipotential neoplasm. We chose the descriptive term "chordoma with malignant spindle cell component" in the sense that high malignant sarcomatous components exists in conjunction with chordomas in the primary tumor and the local recurrence. A review of literature is undertaken chronicling the documented associations of chordoma and sarcoma, followed by a discussion of the various causes proposed to explain this phenomenon.