A40-25 Recurrent Infections and Management Challenges in a Lung Transplant Recipient With Hermansky-Pudlak Syndrome (HPS): A Case Report

Introduction Hermansky-Pudlak syndrome (HPS) is a rare genetic disorder characterized by oculocutaneous albinism, platelet dysfunction, and progressive interstitial lung disease that causes severe pulmonary fibrosis, often necessitating a lung transplant. Therefore, HPS poses unique challenges, including a bleeding predisposition and susceptibility to post-lung transplant infections. Cytomegalovirus (CMV) infection is a ubiquitous risk in seropositive lung transplant recipients, causing chronic lung allograft dysfunction (CLAD) and increased mortality if not effectively managed. Clinical History A 33-year-old female with HPS-related pulmonary fibrosis underwent a left single-lung transplant. Her pre-transplant course was complicated by ARDS, necessitating VV-ECMO as a bridge to transplant. She was a CMV seropositive recipient at the time of transplant, initiating standard antiviral prophylaxis. Her post-transplant course was complicated by the decline in pulmonary function and multiple recurrent bacterial, fungal, and viral infections. Chest CT revealed new ground-glass opacities in her transplanted lung. It was noted that she did not take the prescribed valganciclovir, and a CMV PCR test was positive at 35,000 copies/mL. Concern for CMV disease prompted her urgent referral to the emergency department. Radiographic Figure Pre-transplant chest CT scan of this HPS patient demonstrates extensive bilateral reticulation, diffuse interlobular septal thickening, honeycombing, and significant loss of normal lung parenchyma. Discussion This patient’s case highlights the significant challenges of managing lung transplant recipients, especially those with underlying genetic conditions like HPS. CMV disease is a constant threat for seropositive recipients, who are at high risk for viral reactivation. The profound increase in this patient’s CMV viral load after she stopped taking the antiviral medication underscores the critical importance of strict adherence to antiviral drugs. This adherence is crucial for preventing CMV replication, which can cause direct organ damage and increase the risk of other infections and CLAD. Effective management strategies should include: 1. Strict Medication Adherence Monitoring: Continuously monitor patient compliance with immunosuppressants and prophylactic antivirals. Patient education and pharmacist involvement are essential. 2. Early Recognition of CMV Reactivation: Regularly monitor CMV viral loads, particularly after any changes in the patient’s clinical status. Promptly initiate the antiviral therapy if needed to prevent the progression to overt CMV disease. 3. Multidisciplinary Approach: A collaborative approach involving transplant pulmonologists, infectious disease specialists, radiologists, and pharmacists is vital to differentiate rejection, infection, and other causes of allograft injury. 4. Consider HPS-Specific Vulnerabilities: The underlying HPS may contribute to unique immunological susceptibilities that warrant further research and tailored management. This abstract is funded by: None