Clinical outcomes and survival following lung transplantation in patients with pulmonary Langerhans cell histiocytosis. Issue 6 (12th August 2019)

• Record Type: Journal Article
• Authors: Wajda, Nikolai; Zhu, Zheng; Jandarov, Roman; Dilling, Daniel F.; Gupta, Nishant
• Is Part Of: Respirology. Volume 25:Issue 6(2020)
• Page Start: 644
• Page End: 650
• Publication Date: 2019-08-12
• Subjects: bronchiolitis obliterans -- Langerhans cell histiocytosis -- mortality -- pulmonary Langerhans cell histiocytosis -- pulmonary hypertension; Respiratory organs -- Diseases -- Periodicals; Respiratory organs -- Periodicals; 612.2
• DOI: 10.1111/resp.13671
• Languages: English
• ISSNs: 1323-7799

Abstract

ABSTRACT: Background and objective: Disease‐specific outcomes following lung transplantation (LT) in patients with pulmonary Langerhans cell histiocytosis (PLCH) are not well established. We queried the Organ Procurement and Transplantation Network database to identify adult PLCH patients who had undergone LT in the United States. Methods: Overall survival data were analysed with Kaplan–Meier curves. Cox proportional hazard model was used to determine the effect of demographic, clinical and physiological variables on post‐transplant survival. Results: A total of 87 patients with PLCH underwent LT in the United States between October 1987 and June 2017, accounting for 0.25% of the total LT during this period. The mean age at LT for PLCH patients was 49 years (range: 19–67 years), with a near equal gender distribution. Bilateral sequential LT was performed in 71 patients (82%). Pulmonary hypertension was present in 85% of patients, with a mean pulmonary artery pressure of 38.5 ± 14.1 mm Hg. The mean pre‐transplant forced expiratory volume in 1 s (FEV1 ) was 41 ± 21% predicted and the mean 6‐min walk distance was 221 ± 111 m. Median post‐LT survival for PLCH patients was comparable to patients with other lung diseases (5.1 vs 5.5 years, P = 0.76). The actuarial Kaplan–Meier post‐LT survival for PLCH patients was 85%, 65%, 49% and 22% at 1, 3, 5 and 10 years, respectively. Female sex (hazard ratio (HR): 0.40, 95% CI: 0.22–0.72), pre‐transplant serum bilirubin (HR: 1.66, 95% CI: 1.23–2.26) and serum creatinine (HR: 4.03, 95% CI: 1.01–14.76) were independently associated with post‐LT mortality in our cohort. Conclusion: Post‐LT survival in patients with PLCH is similar to patients with other lung diseases and is significantly affected by patient gender. Abstract : We studied post‐lung transplantation survival and clinical outcomes in patients with pulmonary Langerhans cell histiocytosis (PLCH) from a national transplantation database in the United States. Median post‐transplant survival in patients with PLCH is approximately 5 years, similar to other chronic lung diseases undergoing transplantation, and significantly affected by patient gender.