Recurrence of lymphangioleiomyomatosis: Nine years after a bilateral lung transplantation

Table 1 Serial pulmonary functions in a lung transplant recipient for lymphangioleiomyomatosis

	PreTx-1999	PostTx-2000	2009	2011	2013	2014
FVC	0.81 (27%)	1.70 (57%)	2.06 (71%)	1.90 (80%)	1.83 (79%)	1.76 (77%)
FEV1	0.26 (10%)	1.39 (56%)	1.36 (59%)	1.33 (71%)	1.12 (62%)	1.0 (56%)
FEV1/FVC	32.1 (39%)	81.6 (100%)	65.7 (83%)	69.9 (89%)	61.2 (78%)	57.1 (73%)

FVC: Forced vital capacity; FEV: Forced expiratory volume.

Table 2 Summary of cases with recurrence of lymphangioleiomyomatosis following lung transplantation

Ref.	No. of patients	Type oftransplant	Age at transplantation(yr)	Donor	Post-transplant immunosuppressive drugs	Post-transplant complications	Outcomes
O'Brien et al[5]	1	Single right	NA	NA	NA	NA	NA
Bittmann et al[8,9]	1	Single right	34	Male Cadaveric	NA	Pneumothorax	Survival 2 yr COD: pneumothorax and hypoxemia
Karbowniczek et al[1]	1	Single right	42	Male cadaveric	Cyclosporine, Azathioprine, Prednisone	Chylous pleural effusion	Survival 2 yr COD: Aspergillus pneumonia,
							Recurrence of LAM was confirmed on autopsy
Chen et al[7]	1	Bilateral Living-donor lobar	23	Mother and sister	NA	Massive chylous pleural effusion and ascites	Not known, but she was diagnosed with recurrence of LAM in left lung 2 yr after transplantation due to characteristics cystic changes and pathological confirmation
Sugimoto et al[6]	1	Bilateral Living-donor lobar	23	Brother	Tacrolimus, Prednisone	Un-eventful course	Dyspnea and pleural effusion following 5 yr post-transplant, sirolimus 1-2 mg/d helped resolve pleural effusion and improved lung function and symptoms
Benden et al[10]	4	NA	NA	NA	Cyclosporine, Tacrolimus, Prednisone, Azathioprine	Surgical complications, respiratory tract infections, pneumothorax, pulmonary embolism	Not specified for recurrence of LAM, 5 yr survival was estimated to be 34%

NA: Not available; COD: Cause of death; LAM: Lymphangioleiomyomatosis.