Gene/Protein Disease Symptom Drug Enzyme Compound
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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)

A 51-year-old female with acute myeloid leukemia was admitted to our hospital in December 2001. Though she had undergone two courses of induction chemotherapy (idarubicin hydrochloride + cytarabine), she failed to achieve a complete remission. In April 2002, while in non-complete remission, she subsequently underwent total body irradiation (TBI) and treatment with cyclophosphamide (CY) and etoposide (VP-16) before receiving an allogeneic peripheral blood stem cell transplant from her HLA-identical brother. For graft-versus-host disease (GVHD) prophylaxis, she was given tacrolimus and methotrexate. The infused CD34 positive cells provided 8.1 x 10(6) cells per kg. Engraftment was obtained on post-transplant day 14, and there was no evidence of clinical acute GVHD. The use of tacrolimus was discontinued on post-transplant day 60. As there was no occurrence of clinical acute GVHD, the patient received a donor lymphocyte infusion (CD3 cells 0.57 X 10(7) cells per kg) on post-transplant day 105. On day 132, however, she complained of coughing and fever, and on day 135, she was admitted to our hospital again for dyspnea. A CT scan demonstrated ground-glass opacity in the right pulmonary lobe. After considering her clinical course, symptoms, blood gas, CT scans, etc., we suspected interstitial pneumonia. The dyspnea progressively worsened, however, and despite the use of mechanical ventilation from day 143, the patient died on day 149. From the day she was admitted till the day she was intubated, she was unable to produce sputum. Autopsy findings revealed yellow-white tracheal pseudomembranes, as well as Aspergillus hyphae in the trachea, bronchus, and bilateral lungs. These findings are characteristic of Aspergillus tracheobronchitis. The clinical course of Aspergillus tracheobronchitis in allogeneic stem cell transplant recipients is, however, different from that of the usual invasive Aspergillus infection, and although Aspergillus tracheobronchitis is a very rare disease, attention should be paid to the possibility of its occurrence.
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PMID:[Acute myeloid leukemia with Aspergillus tracheobronchitis after allogeneic peripheral blood stem cell transplant]. 1551 Aug 28

Invasive filamentous fungal infection (IFFI) is an important cause of mortality in allogeneic hematopoietic stem cell transplant (HSCT) recipients. We reviewed 22 consecutive cases of IFFI in allogeneic HSCT recipients at Roswell Park Cancer Institute. IFFI was diagnosed after neutrophil recovery in 21 patients (95%). All had received corticosteroids within 1 month prior to IFFI diagnosis. Fourteen (64%) presented with dyspnea, and only 7 (32%) were febrile. Aspergillus species were isolated in 18 (82%) cases. Thirty day mortality after IFFI diagnosis was associated with a higher mean daily dose of corticosteroids (P=0.02) and receiving OKT3 (P=0.01) within 1 month prior to IFFI diagnosis and serum creatinine>2 mg/dl at the time of diagnosis (P=0.004). Histopathologic material from biopsy or autopsy was available in 15 patients (68%). In 8 (53%), the predominant lung histopathology was an acellular coagulative necrosis and hyphal angioinvasion was observed in some of these cases. These findings have generally been observed in neutropenic patients but not in non-neutropenic HSCT recipients. The predominance of coagulative necrosis in our series may reflect the high doses of corticosteroids used to treat graft-versus-host disease (GVHD), which may have disabled leukocyte trafficking and hyphal killing.
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PMID:Invasive filamentous fungal infections in allogeneic hematopoietic stem cell transplant recipients after recovery from neutropenia: clinical, radiologic, and pathologic characteristics. 1577 Apr 41

Respiratory complications of bone marrow transplantation comprise the majority of its morbidity and mortality. Obstructive airways disease is the most common noninfectious respiratory complication, usually indicative of obliterative bronchiolitis (OB), which occurs in 9% of allogeneic marrow transplant patients. OB is rarely seen after autologous transplant because chronic graft versus host disease (GVH), the most commonly identified risk factor, does not occur in this setting. Alloreactive immunity is likely the cause, with donor type 2 T-helper (TH2) lymphocytes the primary mediators. OB presents at 6 to 12 months post-transplant with cough and dyspnea. Results of investigations include relatively normal or hyperinflated chest radiographs; thickened, dilated airways; and mosaic attenuation on high-resolution computed tomography (HRCT), and fixed airflow obstruction, hyperinflation, and gas trapping on physiological testing. Bronchoscopy and lavage are performed primarily to exclude infections. Transbronchial biopsy is often nondiagnostic, but because the clinical diagnosis is generally sufficient, surgical biopsy is not usually recommended. Histology reveals lymphocytic bronchiolitis, concentric bronchiolar fibrosis, and bronchiolar obliteration. Corticosteroids remain the mainstay of treatment, which is usually required for 3 to 9 months. Response is generally poor, with mortality between 40 and 100%, and lung function infrequently improves. Stabilization with permanent respiratory impairment is common. Early detection and prompt immunosuppression may improve outcomes.
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PMID:Obliterative bronchiolitis complicating bone marrow transplantation. 1608 71

Paraneoplastic pemphigus is an autoimmune bullous skin disease induced by underlying malignant or benign neoplasias. The diagnostic and immunological criteria of the disease were characterized by Anhalt et al. in 1990. Clinical symptoms are variable, consisting of polymorphous blistering skin eruption and severe, painful mucocutaneous ulcerations. In a subset of patients, only papular lesions develop, resembling lichen planus, or graft-versus-host disease; in some cases blisters may develop later. Severe dyspnea, progressive respiratory failure with clinical features of bronchiolitis obliterans is a rather frequent and severe complication. The diagnosis can be established with direct and indirect immunofluorescent studies and immunoblot analysis. The autoantigens identified to date include cytoplasmic proteins of the plakin gene family: envoplakin (210 kD), periplakin (190 kD), plectin (approximately 500 kD), desmoplakin I (250 kD), desmoplakin II (210 kD) and bullous pemphigoid antigen 1 (230 kD). The desmosomal cadherins: desmogleins 1 and 3, and desmocollins 2 and 3, as well as bullous pemphigoid antigen 2 (180 kD) and an undetermined 170-kD transmembranous antigen are also target autoantigens in the disease. The mortality rate is more than 90 percent. Beside treatment of the underlying tumor, a combination of systemic steroids with immunomodulators, cytostatic drugs, plasmapheresis, plasma exchange, intravenous gammaglobulin, or anti-CD20 monoclonal antibody (rituximab) may be the most appropriate treatment.
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PMID:[Paraneoplastic pemphigus]. 1751 51

We describe the clinical courses and outcomes of allogeneic hematopoietic stem cell transplantation-associated organizing pneumonia (HOP) observed in our institution over the past 20 years. Charts and chest radiographs of 603 allogeneic transplant recipients were retrospectively reviewed for HOP. In total, 12 cases of HOP were identified (2.0%) at a median interval of 148 days after transplantation (range, 53-475 days), presenting with low-grade fever, nonproductive cough and dyspnea at onset. Initial antibiotic treatment did not ameliorate symptoms, but most patients responded well to 0.5-1 mg/kg of prednisolone. HOP flare-up occurred after discontinuing treatment or while tapering doses in 9 of 12 patients, but responded to re-treatment with the initial dose of steroid. Although three patients died, no deaths were attributable to pulmonary failure. The remaining nine patients displayed no relapse of primary disease and 5-year survival rate was 74.1%. Clinical features of the 12 patients were similar in that all underwent irradiation-containing conditioning and most had a prior history of acute graft-versus-host disease (GVHD) and cytomegalovirus (CMV) infection. Furthermore, eight patients had active chronic GVHD at onset of HOP. These findings suggest that factors such as irradiation-containing regimens, previous CMV infection and allogeneic immune reaction may contribute to HOP occurrence.
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PMID:Clinical features of allogeneic hematopoietic stem cell transplantation-associated organizing pneumonia. 1761 18

Engraftment syndrome (ES) has been recognized as an inflammatory condition during neutrophil recovery after hematopoietic stem cell transplantation (HSCT) characterized by noninfectious fever and skin rash. It has been reported to occur frequently after autologous HSCT in children and adults, and has been shown to be an independent risk factor for increased transplant-related mortality (TRM). However, virtually no data exist on its occurrence after allogeneic HSCT in children. To determine incidence, predisposing factors for, and complications of ES in a pediatric transplant cohort, we analyzed 61 consecutive recipients of a myeloablative allogeneic HSCT for the occurrence of ES. Diagnosis of ES was established when children presented with > or =2 of the following symptoms within 7 days before engraftment: (1) fever >38.0 degrees C, (2) skin rash, (3) weight gain and albumin drop, or (4) dyspnea, hypoxia, and pulmonary infiltrates. Incidence of ES in this cohort was 48% (29 of 61). In a univariate analysis, posttransplant granulocyte-colony stimulating factor (G-CSF) administration (P = .02), and high mononuclear cell count (MNC) (P = .002) were identified as significant risk factors predisposing for the development of ES. In a multiple logistic regression analysis, amphotericin B therapy (P = .009) and high MNC (P = .004) were significant explanatory variables for ES risk. There was a slight trend toward a higher rate of chronic GVHD (cGVHD) in patients with ES (P = .11). However, after a median follow-up of 9.5 years overall survival (OS) (P = .53) and TRM (P = .65) did not differ between the 2 groups. ES presenting with fever, rash, weight gain, and pulmonary symptoms should be recognized as a frequent complication of allogeneic HSCT after myeloablative conditioning in children. Treatment with G-CSF, amphotericin B, and a high nucleated cell count of the graft predisposed for the development of ES in this study. OS and TRM in this cohort were not affected by the occurrence of ES.
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PMID:Incidence, predisposing factors, and outcome of engraftment syndrome in pediatric allogeneic stem cell transplant recipients. 1834 86

Patients after organ transplantations are at risk for mycobacteriosis development. Frequency of the mycobacterial infection after bone marrow transplantation (BMT) is not as high as one could expect. It ranges from 0.4 to 4.9%. We present a case of a female patient after allogenic BMT as a treatment of chronic myelogenous leucaemia, with bronchiolitis obliterans as a symptom of graft versus host disease (GvHD), treated with corticosteroids and infected with Mycobacterium avium. She was admitted to the hospital with dyspnoea, cough with large amount of sputum production and subfebrile status. She had partial respiratory insufficiency and obturative disturbances of respiration (FEV(1) 0.67 l i.e. 22% of normal) with decline of VC (2.23 l i.e. 64% of normal). The high-resolution computed tomography (HRCT) revealed multifocal infiltrations and bronchiectases in the upper and middle pulmonary fields, which were absent in the previous HRCT taken 3 years earlier. In the bronchial secretion acid-fast bacilli were found by smear and culture. The isolate was classified as Mycobacterium avium complex (MAC) by high performance liquid chromatography (HPLC). The patient was treated with clarithromycin, ciprofloxacin, isoniazide (INH), ethambutol (EMB), amikacin, but M. avium was still present in the sputum after 3 months. Treatment was continued in her parent hospital, where after a few months her sputum became negative for M. avium. But she died over a year later from progressive respiratory insufficiency in the course of bronchiolitis obliterans. The patient was in the group of high risk for mycobacterial infection development and the course of her illness was typical. We decided however to present the case as the topic seems to be quite neglected in the literature.
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PMID:[Mycobacterial infection caused by Mycobacterium avium in allogenic bone marrow transplant recipient with concomittant bronchiolitis obliterans as a manifestation of graft versus host disease - case report and review of the literature]. 1846 26

Acute eosinophilic pneumonia (AEP) is an acute febrile illness with respiratory impairment, diffuse pulmonary infiltrates, and eosinophilia in bronchoalveolar lavage (BAL) fluid. We report an adult male who developed severe cough and dyspnea with slight fever on day 78 after allogeneic hematopoietic stem transplantation. The symptoms coexisted with skin and gut GVHD. The imaging test demonstrated interstitial infiltrates and BAL analysis revealed marked increase of eosinophils and no sign of infection. We made a diagnosis of AEP and steroid was started. AEP remitted with other GVHD symptoms but exacerbated partially when steroid was decreased. This case suggests a potential link between AEP and GVHD.
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PMID:Acute eosinophilic pneumonia is a non-infectious lung complication after allogeneic hematopoietic stem cell transplantation. 1913 57

Pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by the abnormal accumulation of alveolar surfactant protein in alveolar spaces. We report herein a rare case of myelodysplastic syndrome (MDS-RAEB) complicated by severe PAP, and successful allogeneic bone marrow transplantation (BMT) for both disorders. An unrelated BMT was planned for a 48-year-old male with advanced MDS-RAEB. Just before the initiation of the conditioning regimen for unrelated BMT in March 2007, he developed dyspnea. A diagnosis of PAP was made based on findings of chest X-ray, CT scanning, and the fluid obtained by bronchoalveolar lavage. To improve his dyspnea and improve BMT safety, whole lung lavage (WLL) was performed twice, with the partial improvement of PAP. Unrelated allogeneic BMT was performed in September 2007. We had to perform a third WLL because of the worsening of PAP on day 26 after BMT. Despite many infectious complications after BMT, GVHD was relatively mild. PAP had almost disappeared 6 months after BMT. He was well with favorable hematopoiesis 20 months after the BMT without any specific treatment. There has been no report of an MDS patient with PAP in whom 3 WLL procedures were performed before and after allogeneic BMT.
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PMID:Successful allogeneic bone marrow transplantation for myelodysplastic syndrome complicated by severe pulmonary alveolar proteinosis. 1969 50

Toxicity-reduced conditioning is a curative treatment option for medically compromised or elderly patients ineligible for myeloablative hematopoietic cell transplantation (HCT). The aim of this study was to detect prognostic factors for overall survival (OS) and to evaluate quality of life (QOL) in a large homogeneous cohort of 160 consecutive patients aged > or =60 years treated with allogeneic HCT. We evaluated age, sex, performance status, comorbidities, pulmonary function, lactic dehydrogenase concentration, type of donor, disease status, CD34(+) cells transplanted, cytomegalovirus status, time from diagnosis to HCT, and the development of acute and chronic graft-versus-host disease (GVHD). All patients who survived for > or =6 months (n = 79) were asked to complete a QOL survey. All patients (median age, 64.7 years; range, 60.1-76 years) received pretransplantation conditioning with fludarabine, BCNU, and melphalan. With a median follow-up of 35 months, the 1-year OS was 62.4% and 3-year OS was 47.4%. Multivariate analysis revealed compromised performance status as the most significant negative prognostic parameter for OS (P < .003), whereas male donor (P = .008) and chronic GVHD (P = .024) were associated with better OS. The 89% of survivors who returned the QOL questionnaire rated their global QOL as good-to-excellent despite impaired functional capabilities and such symptoms as fatigue, dyspnea, and loss of appetite. The main prognostic factor was performance status, not age. Our data suggest that toxicity-reduced conditioning offers a chance for enhanced OS with an adequate QOL.
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PMID:Prognostic factor and quality of life analysis in 160 patients aged > or =60 years with hematologic neoplasias treated with allogeneic hematopoietic cell transplantation. 2014 20


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