Lung Transplantation for Lymphangioleiomyomatosis in a Single Japanese Institute, With a Focus on Late-onset Complications

Abstract Background Although late-onset complications are important factors related to inadequate outcomes of lung transplantation (LTx), little is known about them. The results of LTx for lymphangioleiomyomatosis (LAM) patients, which is a large cohort of LTx recipients in Japan, especially with late-onset complications, are reported. Methods Thirteen consecutive LTx cases with LAM at our institute were evaluated, and those with late-onset complications were identified. Results The 5-year survival rate was 69.2%. There were 4 cases with late-onset complications. Case 1: A 35-year-old woman who underwent right single LTx and sustained uncontrollable massive chylous ascites. She underwent placement of a peritoneal-venous shunt, and the ascites was controlled. Unfortunately, she died of small cell cervical cancer (SCCC) 43 months after the LTx. Case 2: A 50-year-old woman who underwent left single LTx had pneumothorax of the native lung 16 months after the LTx. She underwent operative repair of the right lung with a polyglycolic acid (PGA) sheet. She had no recurrence of pneumothorax 1 year after the operation. Case 3: A 33-year-old woman, who underwent left single LTx, had recurrence of LAM in the transplanted lung 2 years after the LTx. She was started on sirolimus. Case 4: A 47-year-old woman, who underwent right single LTx, developed repeated high fevers. She developed an acute abdomen, and swollen subcutaneous lymph nodes were found. After lymph node biopsy, she was diagnosed as having post-transplant lymphoproliferative disorder, and she died 8 months after the LTx. Conclusion It is hoped that these reports and the knowledge gained from them help improve the outcomes of LTx recipients.