Secondary pulmonary alveolar proteinosis following allogenic hematopoietic cell transplant for acute myeloid leukemia

SESSION TITLE: Pulmonary Issues in Transplantation Case Report PostersSESSION TYPE: Case Report PostersPRESENTED ON: 10/19/2022 12:45 pm - 01:45 pmINTRODUCTION: Pulmonary Alveolar Proteinosis (PAP) is a diffuse pulmonary disease caused by disruption of surfactant homeostasis that results in surfactant accumulation in the alveoli. It is classified into primary, secondary, and congenital. Secondary PAP is caused by another disease, among which are hematologic malignancies. We report a case of PAP that developed after treatment of acute myeloid leukemia (AML) with hematopoietic cell transplantation (HCT) which is exceedingly rare.CASE PRESENTATION: A 60-year-old female with medical history of bronchiolitis obliterans and AML treated with an allogeneic HCT complicated by chronic Graft Versus Host Disease mainly affecting the skin who presented with dyspnea on exertion. She had a recent hospitalization with staphylococcus epidermis bacteremia.Her physical examination was remarkable for hypoxemia requiring 2 liters of oxygen to maintain oxygen saturation above 90%, otherwise, her physical examination was unremarkable. Her chest computed tomography (CT) showed [ figure 1] showed peripherally predominant peribronchial patchy groundglass opacities bilaterally.Bronchoscopy with transbronchial biopsies and bronchoalveolar lavage was obtained from the right middle lobe and the right lower lobe [figure 2] which showed periodic acid-Schiff positive proteinaceous materials in the alveoli. Her BAL also revealed Mycobacterium fortuitum which was considered colonization and aspergillus which she was treated for. A diagnosis of PAP was made, and the patient was successfully treated with whole lung lavage, repeated chest CT showed significant resolution of the groundglass opacities [ figure 3].DISCUSSION: PAP results from abnormal surfactant accumulation and dysfunction of alveolar macrophages. It can be primary due to autoimmune disease or secondary to a systematic disease such as hematologic malignancies including myelodysplastic syndrome [1], chronic myeloid leukemia, and AML. HCT has been used in the treatment of PAP with AML. However, on extremely rare occasions, PAP might occur even after AML treatment with HCT. Diagnosis can be made with BAL or lung biopsy, the presence of periodic acid-Schiff positive proteinaceous material from bronchoalveolar lavage is diagnostic.[2]. The etiology of PAP after HCT is yet unknown, in one case report it was hypothesized to occur due to macrophage dysfunction or deficient number of macrophages during granulocytopenia which may lead to surfactant accumulation, or contributed to the donor-host alloimmunity [3]CONCLUSIONS: PAP is a rare pulmonary disease that can result from hematologic malignancies such as AML. Clinicians should be aware of the disease as it can develop even after AML treatment with HCT. Early recognition and treatment can result in a significant decrease in morbidity and mortality.Reference #1: Asai Y, Ouchi H, Ohosima T, Nakano R, Yamano Y, Inoshima I et al. [A case of secondary pulmonary alveolar proteinosis associated with myelodysplastic syndrome, complicated with disseminated M. abscessus infection]. Nihon Kokyuki Gakkai Zasshi 2009; 47: 1120–1125.Reference #2: Cordonnier C, Fleury-Feith J, Escudier E, et al. Secondary alveolar proteinosis is a reversible cause of respiratory failure in leukemic patients. Am J Respir Crit Care Med 1994;149:788–94.Reference #3: Pidala, J., Khalil, F. & Fernandez, H. Pulmonary alveolar proteinosis following allogeneic hematopoietic cell transplantation. Bone Marrow Transplant 46, 1480–1483 (2011).DISCLOSURES: No relevant relationships by Shannon BurkeNo relevant relationships by Huazhang GuoNo relevant relationships by Ahmad SharayahNo relevant relationships by David Stoeckel SESSION TITLE: Pulmonary Issues in Transplantation Case Report Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Pulmonary Alveolar Proteinosis (PAP) is a diffuse pulmonary disease caused by disruption of surfactant homeostasis that results in surfactant accumulation in the alveoli. It is classified into primary, secondary, and congenital. Secondary PAP is caused by another disease, among which are hematologic malignancies. We report a case of PAP that developed after treatment of acute myeloid leukemia (AML) with hematopoietic cell transplantation (HCT) which is exceedingly rare. CASE PRESENTATION: A 60-year-old female with medical history of bronchiolitis obliterans and AML treated with an allogeneic HCT complicated by chronic Graft Versus Host Disease mainly affecting the skin who presented with dyspnea on exertion. She had a recent hospitalization with staphylococcus epidermis bacteremia. Her physical examination was remarkable for hypoxemia requiring 2 liters of oxygen to maintain oxygen saturation above 90%, otherwise, her physical examination was unremarkable. Her chest computed tomography (CT) showed [ figure 1] showed peripherally predominant peribronchial patchy groundglass opacities bilaterally. Bronchoscopy with transbronchial biopsies and bronchoalveolar lavage was obtained from the right middle lobe and the right lower lobe [figure 2] which showed periodic acid-Schiff positive proteinaceous materials in the alveoli. Her BAL also revealed Mycobacterium fortuitum which was considered colonization and aspergillus which she was treated for. A diagnosis of PAP was made, and the patient was successfully treated with whole lung lavage, repeated chest CT showed significant resolution of the groundglass opacities [ figure 3]. DISCUSSION: PAP results from abnormal surfactant accumulation and dysfunction of alveolar macrophages. It can be primary due to autoimmune disease or secondary to a systematic disease such as hematologic malignancies including myelodysplastic syndrome [1], chronic myeloid leukemia, and AML. HCT has been used in the treatment of PAP with AML. However, on extremely rare occasions, PAP might occur even after AML treatment with HCT. Diagnosis can be made with BAL or lung biopsy, the presence of periodic acid-Schiff positive proteinaceous material from bronchoalveolar lavage is diagnostic.[2]. The etiology of PAP after HCT is yet unknown, in one case report it was hypothesized to occur due to macrophage dysfunction or deficient number of macrophages during granulocytopenia which may lead to surfactant accumulation, or contributed to the donor-host alloimmunity [3] CONCLUSIONS: PAP is a rare pulmonary disease that can result from hematologic malignancies such as AML. Clinicians should be aware of the disease as it can develop even after AML treatment with HCT. Early recognition and treatment can result in a significant decrease in morbidity and mortality. Reference #1: Asai Y, Ouchi H, Ohosima T, Nakano R, Yamano Y, Inoshima I et al. [A case of secondary pulmonary alveolar proteinosis associated with myelodysplastic syndrome, complicated with disseminated M. abscessus infection]. Nihon Kokyuki Gakkai Zasshi 2009; 47: 1120–1125. Reference #2: Cordonnier C, Fleury-Feith J, Escudier E, et al. Secondary alveolar proteinosis is a reversible cause of respiratory failure in leukemic patients. Am J Respir Crit Care Med 1994;149:788–94. Reference #3: Pidala, J., Khalil, F. & Fernandez, H. Pulmonary alveolar proteinosis following allogeneic hematopoietic cell transplantation. Bone Marrow Transplant 46, 1480–1483 (2011). DISCLOSURES: No relevant relationships by Shannon Burke No relevant relationships by Huazhang Guo No relevant relationships by Ahmad Sharayah No relevant relationships by David Stoeckel