ABSTRACT
Purpose: This study correlates lung transplantation outcomes with pathologic diagnoses of explants and surveillance transbronchial allograft biopsies. Methods: A retrospective IRB approved review of archival material from explanted lung recipients from our Department of Pathology database during the period 2013- 2016 was performed. Demographic information, explant diagnoses, relevant clinical history, chronology of significant clinical events, and cause of death were retrieved. The results of surveillance allograft biopsies and serum donor specific antigen (DSA) antibodies were also reviewed. Log rank test was used to determine differences in survival among various groups. Results: 202 patients with end-stage lung disease underwent lung transplant during the 4-year study period. The most common explant diagnoses were emphysema (n=63, 31.2%), usual interstitial pneumonia (UIP;n=62, 30.7%), and cystic fibrosis (CF;n=22, 11%). Eighty-eight (43.6%) recipients died during the study period. The most common causes of death were chronic lung allograft dysfunction (n=25, 28.4%), malignancy (n=18, 20.5%), and infection (n=17, 19.3%). Three deaths were related to Coronavirus Disease 2019 (COVID-19). The overall survival rates at 3-year and 5-year were 74.3%, and 62.9%, respectively. Although overall survival rates did not correlate with explant diagnoses, patients with UIP and non-specific interstitial pneumonia (NSIP) tended to have worse outcomes than those with emphysema and CF, with lower survival rates in the first year post-transplant. Allograft rejection was identified in 139 patients (68.8%). Most cases of rejection showed acute cellular rejection (ACR, n=126), while antibody-mediated rejection (AMR) was seen in 29 patients, and a combination of ACR and AMR was found in 15 patients. Patients with early ACR (≤ 90 days post- transplant, n=80) had a lower overall survival rate compared to those with late ACR (> 90 days post-transplant, n=46) with a median survival of 68 vs. 88 months, respectively p<0.05 (Figure 1). In addition, patients with AMR alone tended to have lower survival than patients with both ACR and AMR (median survival 48 vs. 68 months), but not significant (p=0.07). Conclusions: This study demonstrates chronic obstructive pulmonary disease (COPD) as the most common indication for lung transplantation in our cohort. Early ACR was associated with adverse outcome by having lower overall survival rate. (Table Presented).
ABSTRACT
Lymphangioleiomyomatosis (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.