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Query: UMLS:C0018133 (graft-versus-host disease)
18,032 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two patients are described with clinical and radiographic bronchiectasis which occurred after allogeneic bone marrow transplantation for haematological malignancy. Both had evidence of chronic graft versus host disease in other organs. Increased immunosuppression with corticosteroids resulted in clinical response, although both patients persisted with chronic mucopurulent sputum production and one had progressive airflow obstruction. Bronchiectasis may be an under-recognised manifestation of chronic graft versus host disease of the lung.
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PMID:Bronchiectasis in bone marrow transplantation. 919 26

A case of bronchiolitis obliterans after allogeneic bone marrow transplantation (BMT) for acute lymphocytic leukemia in an 18-year-old woman with both acute and chronic graft-versus-host disease is described. About 160 days after BMT she started complaining of a non-productive cough and exertional dyspnea. Pulmonary function testing revealed obstructive respiratory dysfunction. High-resolution CT (HRCT) scan of the lungs showed areas of parenchymal hypoattenuation with air-trapping, which was more emphasized with expiratory HRCT. She had many of the risk factors for bronchiolitis obliterans, such as total body irradiation, pretreatment with cyclophosphamide, chronic graft-versus-host disease and a low IgG level. Her symptoms were progressive and bronchiectasis developed. She died of respiratory failure 3.5 years after BMT. Bronchiolitis obliterans is an important complication of BMT which the clinician must take into account.
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PMID:[A case of bronchiolitis obliterans after allogeneic bone marrow transplantation for acute lymphocytic leukemia]. 1216 64

Search for an etiology of bronchiectasis consists in identifying constitutional or acquired defense mechanisms of the respiratory mucosa. The question is timely because causes change. In developing countries, presumed sequelae of infection account for about 30% of the cases despite vaccination campaigns, control of endemic tuberculosis, and widespread use of antibiotics. Genetic diseases account for 20% of the causes when identified by high-performance prospective diagnostic tests (CFTR mutation). Computed tomography enables the identification of frequent associations between bronchiectasis and rheumatoid disease or ulcerative colitis. Recent diseases such as HIV infection or GVHD can also lead to bronchiectasis. Nevertheless, the cause remains unknown in 30-50% of patients. After a detailed analysis of the clinical presentation and diagnostic criteria specific for each etiology, we propose a two-phase diagnostic procedure. The first step, used for all patients (careful history taking, physical examination, imaging, bronchofibroscopy, limited blood tests) enables detecting localized bronchial obstacles and obvious etiologies (situs inversus of primary ciliary dyskinesia, known systemic disease, HIV...). If the first step is negative, the second phase is oriented by the clinical context. Sequelae of infection (tuberculosis...) in older subjects or migrants, a genetic cause in younger subjects, particularly if there is a familial history and/or infertility, a systemic disease or allergic bronchopulmonary aspergillosis if there is an extra-respiratory context. This etiological search should help improve patient management and provide a better prognosis and prevention of bronchiectasis.
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PMID:[Etiological work-up for bronchectasia in adults]. 1568 8

A 64-year-old woman underwent allogeneic peripheral blood-derived stem cell transplantation for acute lymphocytic leukemia. She complained of dyspnea and was admitted to hospital 116 days after transplantation. Because of positive serum testing for the Aspergillus antigen and antibody, and ground-glass opacity in the right upper lobe on high-resolution computed tomography (HRCT), we made a diagnosis of pulmonary aspergillosis and administered an antifungal agent. Although tests for the Aspergillus antibody became negative and the ground-glass opacities disappeared, her dyspnea persisted. Progressive bronchiectasis was seen on HRCT, predominantly in the lower lobes. A pulmonary function test showed mixed impairment. We made a diagnosis of bronchiolitis obliterans after chronic graft versus host disease (GVHD). Prednisolone and an increased dose of tacrolimus (FK506) were administered, but type II respiratory failure progressed and she died 2 months after admission. On HRCT, each lobe was graded for bronchiectasis using a scale: 0 = normal, 1 = less than 2 x the diameter of an adjacent pulmonary artery, 2 = 2 - 3 x the diameter of an adjacent pulmonary artery, and 3 = more than 3 x the diameter of an adjacent pulmonary artery. A total score was calculated by summing the scores of all the lobes (maximum 15). In this case, the total score increased rapidly from 0 to 13 in 2 months.
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PMID:[Case of bronchiolitis obliterans with rapidly progressive bronchiectasis after allogeneic peripheral blood-derived stem cell transplantation]. 2122 5

Pulmonary pathologists were aware of cases of idiopathic interstitial pneumonia (IIP) that morphologically did not fit Liebow's classification scheme. These cases were labeled as "cellular interstitial pneumonia" or "chronic interstitial pneumonia not otherwise specified." The term nonspecific interstitial pneumonia (NSIP) was first used in relation to a pattern of lung interstitial inflammation seen in association with human immunodeficiency virus (HIV) infection. In 1994 NSIP was used to indicate a group of subacute or chronic interstitial pneumonias characterized morphologically by interstitial inflammation or fibrosis or both, with preservation of the lung architecture and the absence of typical findings for any of the other main categories of IIP (mainly usual interstitial pneumonia, desquamative interstitial pneumonia, and bronchiolitis obliterans organizing pneumonia). Although these patients presented with "nonspecific" lung histology (categorized as cellular and fibrotic variants), and with a broad spectrum of associated clinical conditions, such as connective tissue diseases (CTDs), environmental exposure, and previous acute lung injury, they showed some peculiar clinical aspects, including favorable response to corticosteroid treatment and overall good prognosis.The clinical and radiographic profiles were better defined in the last decade. The NSIP pattern is the histological background of a subacute/chronic interstitial pneumonitis that may be observed in many conditions, including CTD, drug-induced lung disease, hypersensitivity pneumonitis, slowly healing diffuse alveolar damage (DAD), relapsing organizing pneumonia, occupational exposure, immunodeficiency (mainly HIV infection), graft versus host disease (GVHD), familial pulmonary fibrosis, immunoglobulin G4 (IgG4)-related sclerosing disease, with or without overlap features with Rosai-Dorfman disease, multicentric Castleman disease, and myelodysplastic syndrome. Rarely, NSIP is the histology recognized in patients with idiopathic interstitial pneumonitis, in whom efforts to find potential causative exposures are futile. This entity occurs mostly in middle-aged, never-smoker women, with a likely association with an autoimmune background. High-resolution computed tomographic (HRCT) scans typically demonstrate ground-glass attenuation with a bibasilar distribution, or in the fibrotic variant, ground-glass attenuation along with reticular lines and traction bronchiectasis. The prognosis is good compared with idiopathic pulmonary fibrosis (IPF), and therapeutic options include mainly corticosteroids and immunosuppressive agents. Recently a more precise definition of clinical profiles and radiographic findings of idiopathic NSIP allows consideration of less invasive diagnostic procedures (bronchoalveolar lavage, transbronchial lung biopsy). Better understanding of pathogenetic mechanisms might widen the therapeutic horizon giving a role to new therapeutic options in more severe cases.
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PMID:Current status of idiopathic nonspecific interstitial pneumonia. 2300 99

Pulmonary chronic graft-versus-host disease (cGvHD) is one of the most common causes of morbidity and mortality after allogeneic hematopoietic stem cell transplantation (aHSCT). Herein, we describe a patient with severe restrictive lung defect secondary to cGvHD. A 21-year-old male patient was admitted to our pediatric intensive care unit (PICU) with pneumonia and respiratory distress. He had a history of aHSCT for chronic myelogeneous leukemia at the age of 17 years. Six months after undergoing aHSCT, he had developed cGvHD involving skin, mouth, eye, lung, liver, and gastrointestinal tract. At the time of PICU admission he had respiratory distress and required ventilation support. Thorax high-resolution computed tomography was consistent with bronchiolitis obliterans. Although bronchiolitis obliterans is an obstructive lung defect, a restrictive pattern became prominent in the clinical course because of the sclerotic chest wall skin. The activity of cGvHD kept increasing despite the therapy and we lost the patient because of severe respiratory distress and massive hemoptysis secondary to bronchiectasis. In conclusion, pulmonary cGvHD can present with restrictive changes related with the advanced sclerosis of the chest wall skin. Performing a fasciotomy or a scar revision for the rigid chest wall in selected patients may improve the patients ventilation.
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PMID:Advanced sclerosis of the chest wall skin secondary to chronic graft-versus-host disease: a case with severe restrictive lung defect. 2457 53

Objective Air-leak syndrome (ALS) is a life-threatening pulmonary complication following allogeneic bone marrow transplantation (allo-BMT) which is thought to be associated with graft-versus-host disease (GVHD). Recently, it has been reported that pleuroparenchymal fibroelastosis (PPFE) also occurs after allo-BMT and often causes ALS. We sought to extract common features of ALS caused by PPFE after allo-BMT. Methods The clinical data of patients who developed ALS caused by PPFE after undergoing allo-BMT (ALS-PPFE) between April 1996 and December 2007 at our institution were collected and reviewed retrospectively. The clinical findings, radiological and pathological features and treatment outcomes of ALS-PPFE were assessed. Results Five patients who developed ALS had histologically proven PPFE (four men, one woman: median age, 37 years). The age of onset of ALS-PPFE was 13 to 109 months (median, 68.8 months) after BMT. Alkylating agents were used as conditioning chemotherapy for BMT in all patients. Only one patient developed chronic GVHD (limited type). The common radiological findings were subpleural thickening and traction bronchiectasis predominantly in the bilateral upper lung fields. The histological pulmonary specimens showed no findings of bronchiolitis obliterans or GVHD. Immunosuppressive therapy was not effective in any of the cases, and all patients died of respiratory failure with or without lung transplantation. Conclusion ALS-PPFE is an extremely late-onset noninfectious pulmonary complication of allo-BMT. This complication is progressive, resistant to immunosuppressive treatment and has a poor prognosis. No association was found between PPFE and GVHD.
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PMID:Air-leak Syndrome by Pleuroparenchymal Fibroelastosis after Bone Marrow Transplantation. 2678 Oct 7

We describe a case of progressive bronchiectasis resulting in cystic bronchiectasis with regions of mucoid impaction as a manifestation of chronic graft versus host disease as a late complication of allogeneic bone marrow transplantation.
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PMID:Progressive Bronchiectasis as a Manifestation of Chronic Graft Versus Host Disease Following Bone Marrow Transplantation. 2730 8

Introduction. We report a case of a patient with acute myeloid leukaemia whose treatment with bone marrow transplantation (BMT) was followed by chronic graft versus host disease (GVHD) with lung involvement and bronchiectasis. This report illustrates an unusual course of a fast progression of the bronchiectasis due to BMT. Case description. A 33-year-old female was diagnosed with acute myeloid leukaemia. An allogeneic BMT was performed. One month after the transplantation, acute GVHD with skin involvement occurred. Treatment with prednisolone and mycophenolate mofetil (MMF) has been started. Nine months later, the patient was examined by a pulmonologist due to progressive dyspnoea. A pulmonary computed tomography (CT) scan showed normal parenchyma of the lungs and no changes to the bronchi. A CT scan performed 7 months later revealed bronchiectasis for the first time. No clinical response was associated with the treatment and the patient's respiratory status progressively deteriorated. During the final hospitalization, a CT scan performed 1 year later revealed huge cystic bronchiectasis in both lungs. Despite the prophylaxis and treatment of GVHD and aggressive antimicrobial therapy, the patient died one year after the diagnosis of bronchiectasis. Conclusions. This case demonstrates that a fast and fatal course of bronchiectasis, that occurs after BMT, should always be considered as a possible manifestation of chronic graft versus host disease (cGVHD) following allogeneic BMT.
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PMID:A fast and fatal course of bronchiectasis: an unusual rare expression of chronic graft versus host disease. A case report. 2835 92