serval:BIB_E6115BB5F58C
Secondary pulmonary alveolar proteinosis treated by lung transplant: A case report.
10.1016/j.rmcr.2020.101108
000557827800026
32528843
Lawi
D.
author
Dubruc
E.
author
Gonzalez
M.
author
Aubert
J.D.
author
Soccal
P.M.
author
Janssens
J.P.
author
article
casereport
2020-05-30
Respiratory medicine case reports
2213-0071
2213-0071
journal
30
101108
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.
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
eng
60_published
true
peer-reviewed
Publication types: Case Reports
Publication Status: epublish
University of Lausanne
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