Lung transplantation after allogeneic stem cell transplantation : a pan-European experience

Late-onset noninfectious pulmonary complications (LONIPCs) affect 6% of allogeneic stem cell transplantation (SCT) recipients within 5 years, conferring subsequent 5-year survival of 50%. Lung transplantation is rarely performed in this setting due to concomitant extrapulmonary morbidity, excessive immunosuppression and concerns about recurring malignancy being considered contraindications. This study assesses survival in highly selected patients undergoing lung transplantation for LONIPCs after SCT. SCT patients undergoing lung transplantation at 20 European centres between 1996 and 2014 were included. Clinical data pre- and post-lung transplantation were reviewed. Propensity score-matched controls were generated from the Eurotransplant and Scandiatransplant registries. Kaplan-Meier survival analysis and Cox proportional hazard regression models evaluating predictors of graft loss were performed. Graft survival at 1, 3 and 5 years of 84%, 72% and 67%, respectively, among the 105 SCT patients proved comparable to controls (p=0.75). Sepsis accounted for 15 out of 37 deaths (41%), with prior mechanical ventilation (HR 6.9, 95% CI 1.0-46.7; p Lung transplantation outcomes following SCT were comparable to other end-stage diseases. Lung transplantation should be considered feasible in selected candidates. No SCT-specific factors influencing outcome were identified within this carefully selected patient cohort.Peer reviewe

Atteinte obstructive des voies aériennes inférieures au cours du syndrome de Gougerot-Sjögren primitif (étude rétrospective dans un centre national de référence des maladie auto-immunes systèmiques rares)

STRASBOURG-Medecine (674822101) / SudocSudocFranceF

Intérêt de la chirurgie de kystes pulmonaires liés à des dépôts de chaînes légères

INTRODUCTION : La maladie des dépôts de chaînes légères d’immunoglobuline est une entité anatomoclinique entraînant, dans de rares cas, une destruction kystique du poumon. OBSERVATION : Nous rapportons le cas d’une patiente de 62 ans, suivie dans le cadre d’une gammapathie monoclonale de signification indéterminée, chez qui, suite à l’apparition d’une dyspnée croissante, des lésions pulmonaires kystiques diffuses sont découvertes au scanner thoracique. Une résection par thoracoscopie du lobe inférieur droit où prédominait l’atteinte kystique a permis le diagnostic histologique de maladie des dépôts de chaînes légères kappa non amyloïdes. La chirurgie a également eu un effet de réduction de volume pulmonaire s’accompagnant d’une amélioration clinique et fonctionnelle via l’amélioration de la ventilation des segments voisins. CONCLUSION : Cette observation de maladie des dépôts de chaînes légères avec atteinte kystique pulmonaire illustre les potentiels bénéfices cliniques et fonctionnels observés après chirurgie de réduction pulmonaire.[Effect of surgery of pulmonary cysts related to immunoglobulin light chain deposits] INTRODUCTION: Light chain deposition disease is a rare anatomo-clinical disorder, which rarely leads to cystic lung destruction. CASE REPORT: We report the case of a 62years old female patient with a history of a monoclonal gammopathy of unknown significance who developed progressive dyspnea. Thoracic CT-scan demonstrated a diffuse pulmonary cystic disorder with predominance in the right lower lobe. Thoracoscopic surgical resection of that lobe led to a diagnosis of non-amyloid kappa light chain deposits. Surgery also resulted in a lung volume reduction effect with clinical and functional benefits related to improved ventilation of adjacent segments. CONCLUSION: This report of pulmonary cystic disorder related to a light chain deposition disease highlights the potential clinical and functional benefits observed after lung volume reduction surgery

Clinical impact of TERT somatic mutation in telomerase-related gene mutation carriers after lung transplantation

International audienceAlmost 25% of patients with pulmonary fibrosis referred for lung transplantation have a germline rare variant of a telomere-related gene. Acquired TERT promoter mutations may counterbalanced the germline defect and reduce the risk of hematological complications in this population. In a series of 34 patients with a germline telomere-related gene mutation who underwent lung transplantation, 12 (35%) patients had at least 1 acquired TERT promoter mutation. Six patients presented myelodysplasia before lung transplantation, with no difference between patients with and without an acquired TERT promoter mutation. After lung transplantation, myelodysplasia developed in only 1 of 8 patients with an acquired TERT promoter mutation versus 7 of 18 patients without a mutation. Survival did not differ between patients with and without an acquired mutation. The presence of an acquired TERT promoter mutation could be associated with reduced hematological complications after transplantation and with better outcome in telomere-related gene mutation carriers but requires further study. (C) 2022 International Society for Heart and Lung Transplantation

Clinical impact of TERT somatic mutation in telomerase-related gene mutation carriers after lung transplantation

International audienceAlmost 25% of patients with pulmonary fibrosis referred for lung transplantation have a germline rare variant of a telomere-related gene. Acquired TERT promoter mutations may counterbalanced the germline defect and reduce the risk of hematological complications in this population. In a series of 34 patients with a germline telomere-related gene mutation who underwent lung transplantation, 12 (35%) patients had at least 1 acquired TERT promoter mutation. Six patients presented myelodysplasia before lung transplantation, with no difference between patients with and without an acquired TERT promoter mutation. After lung transplantation, myelodysplasia developed in only 1 of 8 patients with an acquired TERT promoter mutation versus 7 of 18 patients without a mutation. Survival did not differ between patients with and without an acquired mutation. The presence of an acquired TERT promoter mutation could be associated with reduced hematological complications after transplantation and with better outcome in telomere-related gene mutation carriers but requires further study. (C) 2022 International Society for Heart and Lung Transplantation

Notions de radiobiologie et profession TRM = Begriffe der Radiobiologie und des Berufs der Fachperson für medizinisch-technische Radiologie

La radiobiologie ayant un lien direct avec la pratique des Techniciens en Radiologie Médicale (TRM), nous mettrons en évidence les intérêts des recherches dans ce domaine pour notre profession.Da die Radiobiologie in engem Zusammenhang mit dem Beruf der Fachperson für medizinisch-technische Radiologie steht, zeigen wir die Bedeutung von Forschungen in diesem Bereich für unseren Beruf auf

A Nonsmoker Man in His 40s With a Diagnosis of Genetic-Related Idiopathic Pulmonary Fibrosis (Surfactant-Protein C Gene Mutation)

International audienceA nonsmoker man in his 40s underwent bilateral lung transplantation with a referral diagnosis of genetic-related idiopathic pulmonary fibrosis (IPF). The patient had no medical history in childhood and early adulthood, nor was there a family history of IPF. His nonsmoker father presented with lung cancer at 59 years of age. The patient was a professional brass instrument player; he had started playing at 9 years of age, and he was recently playing 3 to 4 h per day. He had a 7-year clinical history of chronic cough and shortness of breath. Bilateral fine crackles were present at clinical examination. There was no digital clubbing. Data had been collected since 2015: no clinical or immunologic signs of connective tissue disease were evident, including autoantibodies for myositis or anti-synthetase syndrome. Chest radiograph showed diffuse interstitial lung disease. Results of pulmonary function tests yielded a restrictive pattern with decreased FVC and decreased total lung capacity (69% and 47% of predicted, respectively). The FEV1/FVC ratio was 86%, and carbon monoxide transfer coefficient was 36% of predicted. BAL cellular analysis consisted of macrophages (66%), lymphocytes (19%; CD4+/CD8+ ratio, 0.16), neutrophils (10%), and eosinophils (5%)

Proteomic evidence of specific IGKV1-8 association with cystic lung light chain deposition disease

International audienc

A 12-week combination of clarithromycin and prednisone compared to a 24-week prednisone alone treatment in cryptogenic and radiation-induced organizing pneumonia

International audienceBackground Some data suggest that anti-inflammatory macrolides may be effective to treat organizing pneumonia (OP) and prevent relapses, but no formal comparison with prednisone alone is available. To explore this issue, we retrospectively compared the efficacy of a 12-week combined regimen of clarithromycin and prednisone with a 24-week prednisone alone regimen in OP. Methods A standard 12-week regimen of combined clarithromycin and prednisone was designed for the treatment of cryptogenic or radiation-induced OP, aiming at reducing the cumulated prednisone dose and the relapse rate. Its use was left to the discretion of the treating physicians, members of the Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires. Data were compared to a historical control group treated with a standard 24-week prednisone alone regimen. Results 16 patients were treated with combined therapy and 21 with prednisone alone. Complete radiological remission was achieved in 63% of the combined therapy group and 81% of the prednisone alone group (p=0.38). Symptomatic relapses occurred in 81% of the combined therapy group, and 52% of the prednisone alone group (p=0.14). No side effect of clarithromycin was reported. Conclusions In patients with cryptogenic or radiation-induced OP, a 12-week regimen of clarithromycin and prednisone showed no benefit on remission rate and relapse rate as compared to a 24-week prednisone only regimen

Primary Hepatic Lymphoma After Lung Transplantation: A Report of 2 Cases

International audienceBackground. Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of non -Hodgkin lymphoma in the posttransplant setting. Treatment is based on chemotherapy; surgery is still debated and should be performed in very select cases. Methods. We observed 2 patients out of 300 who underwent lung transplantation in the Nouvel Hopital Civil between 2013 and 2019 with primary hepatic lymphoma. Chemotherapy with a rituximab-cyclophosphamide, hydroxydaunorubicin, vincristine, prednisone protocol was performed in all patients. Mycophenolate mofetil was interrupted before treatment, and everolimus was introduced after chemotherapy by associating tacrolimus withdrawal. Results. One patient showed complete remission; after 7 years, no recurrence has been noticed. The second is still undergoing chemotherapy with no signs of disease progression. Conclusions. DLBCL risk is higher in solid organ transplant recipients than in the general population. Primary hepatic lymphoma diagnosis is often difficult and based on histologic findings after initial clinical and radiological suspicion of primary or secondary liver neoplasia. Diagnosis is challenging because no clinical, radiological, or biological features exist. Biopsy is always indicated for histologic confirmation. Chemotherapy is the mainstay of therapy, but surgery may be indicated in very select patients