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

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Version: Final published version
License: CC BY-NC-ND 4.0
Serval ID
serval:BIB_E6115BB5F58C
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Institution
Title
Secondary pulmonary alveolar proteinosis treated by lung transplant: A case report.
Journal
Respiratory medicine case reports
Author(s)
Lawi D., Dubruc E., Gonzalez M., Aubert J.D., Soccal P.M., Janssens J.P.
ISSN
2213-0071 (Print)
ISSN-L
2213-0071
Publication state
Published
Issued date
30/05/2020
Peer-reviewed
Oui
Volume
30
Pages
101108
Language
english
Notes
Publication types: Case Reports
Publication Status: epublish
Abstract
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.
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.
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
Pubmed
Web of science
Open Access
Yes
Create date
16/06/2020 8:31
Last modification date
21/11/2022 8:26
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