Pulmonary platelet thrombi and vascular pathology in acute chest syndrome in patients with sickle cell disease.

ABSTRACT: A growing body of evidence suggests a role for platelets in sickle cell disease (SCD). Despite the proinflammatory, occlusive nature of platelets, a role for platelets in acute chest syndrome (ACS), however, remains understudied. To provide evidence and potentially describe contributory factors for a putative link between ACS and platelets, we performed an autopsy study of 20 SCD cases-10 of whom died from ACS and 10 whose deaths were not ACS-related. Pulmonary histopathology and case history were collected. We discovered that disseminated pulmonary platelet thrombi were present in 3 out of 10 of cases with ACS, but none of the matched cases without ACS. Those cases with detected thrombi were associated with significant deposition of endothelial vWF and detection of large vWF aggregates ad

SUBMITTER: Anea CB 

PROVIDER: S-EPMC4724297 | biostudies-literature | 2016 Feb

REPOSITORIES: biostudies-literature