Case reportRegression of Pulmonary Lymphangioleiomyomatosis (PLAM)-associated Retroperitoneal Angiomyolipoma Post–Lung Transplantation With Rapamycin Treatment
Section snippets
Case Report
A 34-year-old woman with PLAM was referred to our unit for LTx in 2003. PLAM was diagnosed after cholecystectomy, based on a chest CT scan performed after suspicion of aspiration pneumonia. From diagnosis to transplantation, she experienced a rapid deterioration with hypoxemic respiratory failure, progressive dyspnea and significant exercise limitation with associated oxygen desaturation.
CT scan of the abdomen was reported as normal pre-Tx, although it was not reviewed at our unit. She was
Discussion
We contend that the introduction of low-dose rapamycin produced a significant reduction in size of the retroperitoneal angiomyolipoma in this patient. We did not significantly alter the cyclosporine levels, but azathioprine was ceased. At our institution, we have an inadvertent “control period” of 20 months, including 14 months of triple immunosuppression with full-dose cyclosporine, azathioprine and prednisolone. During this period the retroperitoneal tumor had not altered significantly in
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