scholarly journals Relapse of Lymphangioleiomyomatosis Five Years after Bilateral-Lung Transplantation

2021 ◽  
Vol 24 (9) ◽  
pp. 701-703
Author(s):  
Jalal Heshmatnia ◽  
Maryam Sadat Mirenayat ◽  
Mitrasadat Rezaei ◽  
Felix Bongomin ◽  
Mehrdad Bakhshayeshkaram ◽  
...  

Pulmonary lymphangioleiomyomatosis (LAM) is an uncommon disease principally affecting women during childbearing years and eventually leading to progressive respiratory failure. Lung transplantation is a viable option for patients with end-stage disease. LAM-related complications remain common, but recurrence of LAM following allograft transplantation is rare. We present a 25-year-old woman who presented with progressive dyspnea five years after bilateral lung transplantation for end-stage LAM. Histological examination of transbronchial lung biopsy sample confirmed recurrent LAM. We changed cyclosporine to sirolimus and she is currently being considered for re-transplantation.

2021 ◽  
Vol 4 (1) ◽  
Author(s):  
Sakda Sathirareuangchai ◽  
Jenny L. Weon ◽  
Suzanne Tintle ◽  
Kiran Batra ◽  
Shirley X. Yan ◽  
...  

AbstractLymphangioleiomyomatosis (LAM) is a rare neoplastic disease of the lung with a characteristic feature of diffuse cystic changes in bilateral lungs. Lung transplantation is considered to be one of the effective treatments in end stage disease. Patients with LAM who underwent lung transplant tend to have more favorable outcome compared to other end stage lung diseases. We report a case of a female patient who was diagnosed with LAM and received bilateral lung transplantation at 45 years of age. Subsequent allograft biopsies were significant for mild acute cellular rejection (Grade A2), for which the immunosuppressive regimen was adjusted accordingly. At 7 years post-transplant, she presented with shortness of breath, cough, and fatigue, and diagnosed with a viral infection. Her chest imaging was unremarkable. However, a transbronchial biopsy was performed to rule out rejection and revealed foci of spindle cells proliferation, with positive HMB-45 and smooth muscle actin immunohistochemical studies, confirming the diagnosis of recurrent LAM. After she was discharged, she was re-admitted 1 week later with severe COVID-19. Her clinical course was complicated by acute respiratory distress syndrome, respiratory failure, and gastrointestinal hemorrhage. The patient passed away on day 36 of hospital stay. Autopsy was requested and confirmed the pathology of recurrent LAM and diffuse alveolar damage from COVID-19.


2021 ◽  
Vol 30 (3) ◽  
pp. 178-183
Author(s):  
Kate McEwen ◽  
Lyndell Brodie

Lung transplantation is a well-established treatment for a variety of end-stage pulmonary diseases. However, the journey of a lung transplant recipient is complex and multifaceted. Silicosis is a rare indication for lung transplantation, but no other treatment is yet available for this disease in its end stages. This Australian case study presents a 52-year-old man with silicosis who received bilateral lung transplantation. The patient was frequently noncompliant with noninvasive ventilation therapy and experienced the complication of type 2 respiratory failure. Patient education and support provided, particularly around medication management following transplantation surgery, are discussed here. The patient's social situation and its implications for both him and his family are also considered.


2021 ◽  
Vol 40 (4) ◽  
pp. S500
Author(s):  
N. Maniar ◽  
J. Coster ◽  
G. Li ◽  
J. Segraves ◽  
M. Hemmersbach-Miller ◽  
...  

2019 ◽  
Vol 29 (2) ◽  
pp. 115-121 ◽  
Author(s):  
Jawad Salman ◽  
Fabio Ius ◽  
Wiebke Sommer ◽  
Thierry Siemeni ◽  
Felix Fleissner ◽  
...  

Introduction: Lymphangioleiomyomatosis (LAM) is a rare disease in women, leading to progressive deterioration of lung function and respiratory failure. We describe the outcome of patients with end-stage LAM who underwent lung transplantation at our center. Materials and Methods: The records of patients with LAM transplanted at our institution between February 1997 and May 2015 were reviewed retrospectively. Morbidity and mortality were analyzed, and actuarial survival was calculated using Kaplan-Meier methods. The cumulative survival of transplant patients with LAM at our center was compared with survival after transplantation due to different diseases at our center and the results of the International Society for Heart and Lung Transplantation. Quality of life was assessed by a patient self-report at the end of the first postoperative year. Results: During the study period, 25 patients underwent lung transplantation for LAM. All patients were women with a mean age of 50 (9) years. Thirteen patients (52%) had undergone previous thoracotomy. All patients (100%) received bilateral lung transplantation. One (4%) case of in-hospital mortality occurred and 9 (36%) late deaths. Two (8%) cases of late death were due to chronic lung allograft dysfunction. The 1-, 3-, and 5-year survival rates were 92%, 84%, and 76%, respectively. Quality-of-life ratings were above the normal in all eight 36-Item Short Form Health Survey subscales 1 year after transplantation. Conclusions: Lung transplantation offers a valuable therapy for patients with end-stage pulmonary LAM.


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