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Secondary pulmonary alveolar proteinosis treated by lung transplant: A case report.
- Source :
-
Respiratory medicine case reports [Respir Med Case Rep] 2020 May 30; Vol. 30, pp. 101108. Date of Electronic Publication: 2020 May 30 (Print Publication: 2020). - Publication Year :
- 2020
-
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.<br />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.<br />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.<br />Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.<br /> (© 2020 The Authors.)
Details
- Language :
- English
- ISSN :
- 2213-0071
- Volume :
- 30
- Database :
- MEDLINE
- Journal :
- Respiratory medicine case reports
- Publication Type :
- Report
- Accession number :
- 32528843
- Full Text :
- https://doi.org/10.1016/j.rmcr.2020.101108