Case report: Serious unexpected vascular events in two patients with lymphocytic variant hypereosinophilic syndrome

Abstract

BackgroundLymphocytic-variant hypereosinophilic syndrome (L-HES) is a form of reactive hypereosinophilia, most commonly associated with interleukin-5 over-production by clonal, most commonly CD3−CD4+CD2hiCD5hiCD45RO+ T-cells. Patients often present with predominant cutaneous and soft-tissue manifestations, while cardiovascular involvement is uncommon.MethodsWe reviewed the medical files of two L-HES patients followed in our center who developed serious vascular complications and performed a literature review for similar cases.ResultsPatient 1, a 52-year-old female, presented with an ischemic stroke secondary to left middle cerebral artery dissection after 10 years of indolent L-HES. Blood eosinophilia was controlled with oral corticosteroids (OCS), but OCS-tapering attempts with hydroxyurea and pegylated interferon failed, prompting the introduction of mepolizumab with rapid normalization. Patient 2, a 62-year-old female, had been asymptomatic for 10 years without treatment when a NSTEMI occurred, due to coronary artery occlusion secondary to a large cauliflower-aneurysm of the proximal aorta and aneurysmal dilatation of several coronary arteries, requiring semi-urgent surgical management. Aortic wall staining for eosinophil major basic protein showed eosinophils in the adventitia. Blood eosinophilia was controlled with OCS.ConclusionsPatients with apparently clinically benign L-HES may develop arterial complications, consisting in dissection and/or aneurysm dilatation of medium-to-large vessels with serious consequences. The value of performing regular vascular imaging and monitoring during follow-up has yet to be determined.

Hypereosinophilic syndrome with cardiac and cutaneous involvement: Missed treatment opportunity of a case from Ethiopia

“…3 Cardiovascular complications account for significant HES-related morbidity and mortality. [6][7][8][9] In addition to direct eosinophil-mediated damage to the heart, coronary vasospasm, arterial and/ or venous thrombi as well as microvascular damage may occur. 8,10 Diagnosis of HES requires a high index of suspicion and careful evaluation to exclude secondary causes of hypereosinophilia.…”

“…Commonly involved organs include skin, heart, lungs, gastrointestinal tract, and central nervous system 3 . Cardiovascular complications account for significant HES‐related morbidity and mortality 6–9 . In addition to direct eosinophil‐mediated damage to the heart, coronary vasospasm, arterial and/or venous thrombi as well as microvascular damage may occur 8,10 …”