Bilateral Lung Transplantation for Lymphangioleiomyomatosis With Secondary Pulmonary Hemosiderosis: A Case Report.

Abstract

Background: Pulmonary lymphangioleiomyomatosis (LAM) is a rare and progressive disease characterized by abnormal proliferation of smooth muscle-like cells leading to diffuse cystic destruction of the lung parenchyma and respiratory compromise, predominantly affecting young women. Idiopathic pulmonary hemosiderosis (IPH), a rare disorder characterized by recurrent diffuse alveolar hemorrhage and iron deposition in the lungs, may present with nonspecific respiratory symptoms and radiologic findings that can overlap with other diffuse lung diseases, potentially leading to diagnostic confusion. Such overlap may delay accurate diagnosis and appropriate management.

Case presentation: We present the case of a 29-year-old woman with a prior diagnosis of IPH who was listed for lung transplantation. She presented with severe acute respiratory decompensation and required urgent bilateral lung transplantation. Due to intraoperative hemodynamic instability, she underwent the procedure under venoarterial extracorporeal membrane oxygenation (VA-ECMO) support. Histopathological analysis of the explanted lungs revealed pulmonary LAM as the actual underlying condition, not IPH.

Conclusion: This case highlights the diagnostic complexity of rare pulmonary diseases and the potential for misdiagnosis, particularly in advanced stages. It underscores the importance of considering LAM in the differential diagnosis of diffuse lung diseases in young women and demonstrates the critical role of lung transplantation and early multidisciplinary intervention in managing end-stage respiratory failure due to rare etiologies.