Acute Exacerbation of Pleuroparenchymal Fibroelastosis Secondary to Allogenic Hematopoietic Stem Cell Transplantation

Abstract

In this article, we report a case with pleuroparenchymal fibroelastosis (PPFE) following hematopoietic stem cell transplantation (HSCT) that developed acute respiratory failure with new bilateral ground glass opacity, which could not be explained by either a pulmonary infection, drug toxicity or extraparenchymal causes. Although combination therapy with multiple immunosuppressants was transiently effective, the patient died from a recurrent exacerbation. Autopsied lungs demonstrated diffuse alveolar damage superimposed on PPFE. There was no evidence of any coexisting interstitial pneumonia with the usual interstitial pneumonia (UIP) pattern. Our case suggests that acute exacerbation can occur in patients with post-HSCT PPFE, even when a coexisting UIP pattern is absent.

Pleuroparenchymal Fibroelastosis: A Review with a Focus on a Non-Infectious Complications after Hematopoietic Stem Cell Transplant

“…During follow-up, there is a gradual decrease in the FVC and an increase in the RV/TLC ratio in most patients [ 7 , 15 , 25 ]. For some patients, however, this change in pulmonary function testing occurs more rapidly, with a corresponding increase in their symptoms, as would be expected [ 27 , 28 ].…”

Pleuroparenchymal Fibroelastosis and Serositis as Pleural Complications after Hematopoietic Stem Cell and Lung Transplantation

“…Typically repeat pulmonary function tests in most patients show a gradual decrease in the FVC with a corresponding increase in the RV/TLC ratio over time [42,53,71,74]. However, in some patients this change occurs more rapid with a increase in their symptoms and findings of desaturation at rest or with activity [75,76].…”