Secondary pulmonary alveolar proteinosis treated by lung transplant: A case report

David Lawi; Estelle Dubruc; Michel Gonzalez; John-David Aubert; Paola M Soccal; Jean-Paul Janssens

doi:10.1016/j.rmcr.2020.101108

Secondary pulmonary alveolar proteinosis treated by lung transplant: A case report

Respir Med Case Rep. 2020 May 30:30:101108. doi: 10.1016/j.rmcr.2020.101108. eCollection 2020.

Authors

David Lawi¹, Estelle Dubruc², Michel Gonzalez³, John-David Aubert^{4

5}, Paola M Soccal^{1

6}, Jean-Paul Janssens^{1

6}

Affiliations

¹ Division of Pulmonology, Geneva University Hospitals, 1211, Geneva, Switzerland.
² Division of Pathology, Lausanne University Hospital, 1011, Lausanne, Switzerland.
³ Division of Thoracic Surgery, Lausanne University Hospital, 1011, Lausanne, Switzerland.
⁴ Division of Pulmonology, Lausanne University Hospital, 1011, Lausanne, Switzerland.
⁵ Faculty of Medicine, University of Lausanne, Lausanne, Switzerland.
⁶ Faculty of Medicine, University of Geneva, 1211, Geneva, Switzerland.

Abstract

Background: Pulmonary alveolar proteinosis (PAP) is a pulmonary disease characterized by disruption of surfactant homeostasis resulting in its accumulation in the alveoli. PAP is classically classified into three categories (Table 1): 1/primary (or autoimmune) with antibodies targeting the GM-CSF pathway, 2/secondary to another disease, typically a hematologic malignancy, and 3/genetic.

Case-report: A 30 year-old woman received an allogenic hematopoietic stem cell transplantation (HSCT) after treatment for acute myeloid leukemia (AML). Within the first 6 months post HSCT, she developed an ocular, oral, digestive and hepatic graft-versus-host disease associated with a mixed ventilatory defect with a very severe obstructive syndrome and a severe CO diffusion impairment. High resolution computed tomography showed a classical "crazy paving" pattern. Aspect and differential cell count of BAL were normal. All microbiological samples remained culture negative. Histo-pathological analysis of transbronchial biopsies was unremarkable. Because of the severity of the respiratory insufficiency, open-lung biopsy (OBL) could not be performed. Despite multiple immunosuppressive therapies, lung function deteriorated rapidly; the patient also developed an excavated fungal lesion unresponsive to treatment. She underwent a bilateral lung transplant 48 months after HSCT. Histo-pathological analysis of explanted lungs showed obliterative bronchiolitis (OB), diffuse PAP and invasive cavitary pulmonary aspergillosis.

Conclusions: This case illustrates the simultaneous occurrence of OB, PAP and a fungal infection in a 30-year old female patient who underwent HSCT for acute myeloid leukemia (AML). To our knowledge this is the only documented case of PAP associated with OB treated by lung transplantation.

Keywords: AML, Acute myeloid leukemia; BAL, Bronchoalveolar lavage; BLT, Bilateral Lung Transplant; GVHd, Graft-versus-host disease; HRCT, High Resolution Computed Tomography; HSCT, Hematopoietic Stem Cell Transplantation; Invasive pulmonary aspergillosis; Lung transplantation; OB, Obliterative Bronchiolitis; OLB, Open-lung biopsy; Obliterative bronchiolitis; PAP, Pulmonary Alveolar Proteinosis; PFT, Pulmonary Function Tests; Secondary pulmonary alveolar proteinosis; TBB, Transbronchial Biopsy.

Publication types

Case Reports