Transplantation |

Lymphangioleiomyomatosis: Recurrence Following Double Lung Transplantation FREE TO VIEW

Khawaja Zaki, MD; Zahra Aryan; Marie Budev, MD; Olufemi Akindipe, MD; Atul Mehta, MD
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Cleveland Clinic Foundation, Cleveland, OH

Chest. 2014;146(4_MeetingAbstracts):986A. doi:10.1378/chest.1994397
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SESSION TITLE: Transplant Cases

SESSION TYPE: Affiliate Case Report Slide

PRESENTED ON: Sunday, October 26, 2014 at 03:15 PM - 04:15 PM

INTRODUCTION: Lymphangioleiomyomatosis(LAM) is a rare, slowly progressive neoplasm of young women. The condition causes proliferation of abnormal smooth muscles that target lungs, causing cystic destruction and respiratory failure over years. The decline in lung function is approximately 15%/year1. In patients with end-stage LAM, lung transplantation (LT) is a viable option. Due to its systemic nature there is a risk of recurrence in donor’s lung. There are case reports of LAM recurrence in allogeneic single lung transplant(SLT). We describe a case of a recipient of a double lung transplantation(DLT) who developed LAM recurrence nine years post-transplant.

CASE PRESENTATION: 66 year old female underwent DLT for LAM in 1999, treated with prednisone, tacrolimus, mycophenolate. RUL mass and nodules were noticed on CT in 2008, and was diagnosed with Aspergillus, Pseudomonas and MAI infections which were treated. Her DOE persists with gradual decline in her FEV1 from post-transplant baseline of 1.39 to 1.06. There is no radiological or histological evidence of bronchiolitis obliterans neither of rejection (Fig-1). Histopathology revealed inflammation and atypical spindle/LAM cells confirming recurrence of LAM. Immunohistochemical studies for HMB45 were negative due to scant number of LAM cells. To stabilize lung function and improvement of symptoms she is placed on sirolimus2.

DISCUSSION: LAM is a progressive disease. The only viable treatment option so far is lung transplantation until recent success with mTOR inhibitor which has shown stabilization of lung function and improvement in QOL2. LAM recurrence diagnosis is often confused with OBS and high degree of suspicion is required. Despite benign nature, recurrence is likely due to migration of LAM cells3. Migration of LAM cell to allograft in our patient who underwent DLT with no evidence of renal angiomyolipoma adds another challenge in treating these patients.

CONCLUSIONS: Double Lung transplantation in LAM also possesses various challenges with addition of recurrence. It is likely due to migration of LAM cells from an occult source. Benefits of mTOR inhibitors remain to be seen.

Reference #1: Henske EP et al. Lymphangioleiomyomatosis-a wolf in sheep's clothing. J Clin Invest. 2012 Nov 1;122(11):3807-16

Reference #2: McCormack FX et al. Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med. 2011 Apr 28;364(17):1595-606

Reference #3: Karbowniczek M et al. Recurrent LAM after transplantation: genetic analyses reveal a metastatic mech. Am J Respir Crit Care Med. 2003 Apr 1;167(7):976-82

DISCLOSURE: The following authors have nothing to disclose: Khawaja Zaki, Zahra Aryan, Marie Budev, Olufemi Akindipe, Atul Mehta

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