Multiple Segmental Lung Lavages in the Treatment of Secondary Alveolar Proteinosis

Abstract

SESSION TITLE: Pulmonary Manifestations of Systemic Disease 4 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Pulmonary alveolar proteinosis (PAP) is characterized by the accumulation of periodic acid-Schiff (PAS)-positive material in the lungs. We present a case of multiple segmental lung lavages (MSLL) in the treatment of secondary PAP where whole-lung lavage (WLL) was precluded due to severe refractory hypoxemia. CASE PRESENTATION: A 34-year-old man with history of myelodysplastic syndrome (MDS) status post allogeneic bone marrow transplant was admitted to the intensive care unit (ICU) due to acute on chronic hypoxemic respiratory failure. He was afebrile, hemodynamically stable, his oxyhemoglobin saturation was 92% on 10L of oxygen via non-rebreather mask, and coarse breath sounds were present bilaterally. He had a history of Mycobacterium avium complex (MAC) pulmonary disease and a recent bone marrow biopsy showed MDS recurrence with positive familial GATA2 mutation. Computer tomography (CT) of the chest showed diffuse bilateral groundglass and interstitial opacities. Except for sputum culture positive for MAC, his infectious workup was negative. He was intubated and mechanically ventilated. A bronchoalveolar lavage demonstrated a milky fluid sample with proteinaceous sediment positive for PAS, suggesting PAP. MonoMac syndrome1 was suspected given his MAC pulmonary infections, MDS/GATA2 mutation, and PAP. With severe refractory hypoxemia, WLL was precluded and MSLL were considered as an alternative. Initially, the right lower lobe was lavaged with sequential instillations of saline 35mL-aliquots until the fluid became clear. Within 24 hours, FiO2 requirements decreased from ~0.8-1.0 to 0.65. Segmental lavages of the left lower lobe, and the right upper and middle lobes were completed within the next 48 hours. Unfortunately, the patient developed multiple organ system failure (MOSF) and his family opted for comfort cares. DISCUSSION: Secondary PAP can be found in association with MonoMac syndrome. This case was a diagnostic and therapeutic challenge due to the patient’s comorbidities, decreased functional status at baseline, and atypical findings for PAP on CT of the chest. MSLL resulted in decreased oxygen requirements; however, the impact of these interventions on the final outcome was lessened by the development of MOSF in this case. CONCLUSIONS: When severe hypoxemia among patient with PAP precludes WLL as a therapeutic intervention, multiple segmental lung lavages could be an alternative treatment option, or a temporizing method allowing clinical improvement prior to WLL. Reference #1: 1.Blood.2011;118:2656-2658 DISCLOSURE: The following authors have nothing to disclose: Gustavo Cortes Puentes, Darlene Nelson, James Utz No Product/Research Disclosure Information