Hypereosinophilic Syndrome: A Case of Fatal Löffler Endocarditis

Abstract

Hypereosinophilic syndrome (HES) is a rare disorder with unknown global prevalence, barely reported in Hispanic population, and characterized by persistent eosinophilia in association with organ dysfunctions directly attributable to eosinophilic infiltration. Cardiac involvement may be present in 50 to 60% of the patients. This is known as Löffler endocarditis. We present a case of a 36-year-old Hispanic man with signs of heart failure. Laboratory studies showed eosinophilia (23,100/μL). Thoracic computer tomography showed bilateral pleural effusion and a large left ventricular mass. Transthoracic echocardiography showed left ventricle apical obliteration and a restrictive pattern. Pulmonary angiography demonstrated a thrombus in the lingular and middle lobe. Despite treatment, the patient deceased seven days after admission. Autopsy confirmed the diagnosis of Löffler endocarditis.

When a death apparently associated to sexual assault is instead a natural death due to idiopathic hypereosinophilic syndrome: The importance of gamma-hydroxybutyric acid analysis in vitreous humor

“…Podjasek and Butterfield [24], who conducted a retrospective review of the morbidities and causes of death in HES patients at Mayo Clinic, have examined 23 fatal cases and the most common cause of death was cardiac in 65% of cases, whereas pulmonary vasculitis was identified only in one case, making the latter very rare. Literature review also confirms the predominant cardiac involvement (Loffler endocarditis) among HES fatalities with respect to all other causes of death [24,25].…”

Loeffler’s Endocarditis and Hypereosinophilic Syndrome

“…A study using the Surveillance, Epidemiology, and End Results (SEER) databank to estimate the prevalence of this syndrome, showed it to be anywhere from 0.36 to 6.3 per 100,000, with extremely low reports in the Hispanic population. 6,7 While this syndrome is predominantly more common in males than females with a 9:1 ratio, certain subtypes such as the lymphocytic variant and the idiopathic variant suggest an equal distribution between both male and female patients. [8][9][10] The most common ages affected are between 20 and 50 years old, although there have been a few cases of pediatric hypereosinophilia recorded in the literature.…”

“…One of the serious presentations of this disease is when there is an involvement of the cardiac tissue, which occurs in more than 50% of all patients with HES. 7,10,[12][13][14][15] A few risk factors for cardiac involvement in patients with already established HES include HLA-Bw44 positivity, thrombocytopenia, increased vitamin B12, splenomegaly, or FIP1L1-PDGFRA fusion tyrosine kinase. [15][16][17] Patients who are living in regions known for a greater incidence of parasite infections or individuals who are exceedingly atopic may be at greater risk due to secondary activation of eosinophilia.…”

“…Cationic protein has also been noted to play a role by binding to endothelial thrombomodulin, resulting in a hypercoagulable state. 7,14,[18][19][20][21][22][23] Damage to the vascular wall by eosinophils also exposes the subendothelial collagen and von Willebrand factor that leads to a hypercoagulable state. 14,24,25…”

Case report: Different clinical manifestations of the rare Loeffler endocarditis

“…Imatinib is the first choice for treatment. The Loeffler endocarditis originates from idiopathic and secondary eosinophilia, corticosteroids are usually the primary medication ( 17 ). In addition, it is important to save samples for clonality (genetic, flow cytometry) before corticosteroids' initiation, if possible, because corticosteroids often have a very rapid effect, blurring the diagnostic clues.…”