Pulmonary Artery Thrombosis during Acute Chest Syndrome in Sickle Cell Disease

Abstract

PT is not a rare event in the context of ACS and seems more likely in patients with higher platelet counts and lower hemolytic rate during ACS. Patients with sickle cell disease presenting with respiratory symptoms suggestive of ACS may benefit from evaluation for PT.

Lung vaso-occlusion in sickle cell disease mediated by arteriolar neutrophil-platelet microemboli

“…Our finding that pulmonary vaso-occlusion occurs in precapillary pulmonary arterioles is distinct from the current paradigm for systemic vaso-occlusion, which suggests that postcapillary venules are the principal site of vaso-occlusion (1). These new observations of arteriolar pulmonary vaso-occlusion are supported by recent clinical studies that reveal selective occlusion of pulmonary artery branches and arterioles by aggregates of platelets in lung autopsies (27) and CT scans (32) of SCD patients diagnosed with lung injury.…”

Prevalence and risk factors for venous thromboembolism in children with sickle cell disease: an administrative database study

“…42 A high prevalence of PE in patients with SCD or SC trait has been previously noted in adult studies, [43][44][45] and is hypothesized to result from in situ pulmonary artery thrombosis. Interestingly, even though the majority of thrombotic events occurred in the setting of CVLs, VTE was associated with an increased HR of death.…”

Thrombospondin-1 inhibits ADAMTS13 activity in sickle cell disease

“…Critically ill patients with ACS develop thrombocytopenia from platelet consumption 2 and have a high incidence of pulmonary embolism, 3 thereby suggesting that hemostatic activation plays a role in ACS.…”