Hypereosinophilic Syndrome

ABCIC

Case Report

Important Mitral Regurgitation and Ventricular Dysfunction in Hypereosinophilic Syndrome: A Case Report

Marcel Pina Ciuffo et al. Lucas Mori Tais Araújo Ingrid Kowatsch Ana Clara Tude Rodrigues

Introduction

Hypereosinophilic syndrome (HES) represents a group of rare hematologic diseases characterized by an increased number of eosinophils in the peripheral blood. Once activated, these eosinophils can cause damage to host tissues and organs. Patients with HES generally present with dermatological, respiratory, and gastrointestinal signs and symptoms. Involvement of the cardiovascular system is uncommon and can lead to serious consequences such as heart failure, arrhythmias, and thromboembolic phenomena.

This article describes a case of eosinophilic endomyocardial disease, also known as Loeffler syndrome, a manifestation of HES characterized by eosinophil-mediated cardiac damage. Despite adequate treatment and control of the hematologic disease, the patient developed biventricular systolic dysfunction and important mitral regurgitation (MR).

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ABC

Review Article

Cardiac Abnormalities in Hypereosinophilic Syndromes

Viviane Tiemi Hotta et al. Rafael Ruas Nastari Gardênia da Silva Lobo Oishi Alexandre Eiji Kayano … Juliana Alzira Gonzales Oliveira Ruiza Gonçalves Rocha Ana Olga Mocumbi Fernanda Salles Seguro José Eduardo Krieger Fábio Fernandes Vera Maria Cury Salemi

Abstract

Hypereosinophilia (HE) is defined as an eosinophil count exceeding 1500 cells/microL in peripheral blood in two tests, performed with an interval of at least one month and/or anatomopathological confirmation of HE, with eosinophils comprising more than 20% of all nucleated cells in the bone marrow. Hypereosinophilic syndrome (HES) indicates the presence of HE with organ involvement due to eosinophil action, which can be classified as primary (or neoplastic), secondary (or reactive), and idiopathic. Cardiac involvement occurs in up to 5% of cases in the acute phase and 20% of the chronic phase of the disease, ranging from oligosymptomatic cases to fulminant acute myocarditis or chronic restrictive cardiomyopathy (Loeffler endomyocarditis). However, the degree of cardiac dysfunction does not directly correlate with the degree of eosinophilia. The cardiac involvement of HES occurs in three phases: initial necrotic, thrombotic, and finally necrotic. It can manifest as heart failure, arrhythmias, and thromboembolic phenomena. The diagnosis of cardiopathy is based on multimodality imaging, with an emphasis on the importance of echocardiography (echo) as the primary examination. TTE with enhanced ultrasound agents can be used for better visualization, allowing greater accuracy in assessing ventricular apex, and myocardial deformation indices, such as longitudinal strain, may be reduced, especially in the ventricular apex (reverse apical sparing). Cardiac magnetic resonance imaging allows the characterization of subendocardial late gadolinium enhancement, and endomyocardial biopsy is considered the gold standard in diagnosing cardiopathy. Treatment is based on the etiology of HES.

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ABCIC

Case Report

Aortic Valvulitis in Hypereosinophilic Syndrome: A Case Report

Mariano Gaston Bergier et al. Rocío Blanco Santiago Decotto Anibal Arias

Introduction

Hypereosinophilic syndromes are characterized by the sustained overproduction of eosinophils, which can infiltrate various organs and release toxins that damage tissues. Causes of increased eosinophils include 1) primary or neoplastic causes (e.g., stem cell, myeloid, or eosinophil neoplasia, and clonal disorders); 2) secondary or reactive causes (e.g., parasitic infections, solid tumors, and T-cell lymphomas); 3) idiopathic causes: part of specific syndromes (e.g., Churg-Strauss); and 4) hypereosinophilia of unknown significance, when there is no organic damage.

When specifically discussing rheumatic syndromes, the most common one is eosinophilic granulomatosis with polyangiitis, formerly known as Churg-Strauss disease. This vasculitis affects small and medium-sized arteries, primarily affecting the skin and lungs. However, it is a multisystem disorder that can affect kidneys, heart, and nervous system as well. This condition is often associated with chronic rhinosinusitis, asthma, and eosinophilia. ANCA antibodies are usually present, except in cases where the heart is involved. In such cases, ANCA antibodies are usually negative, but eosinophil counts remain elevated. Whenever possible, confirmatory diagnosis should be confirmed through an endomyocardial biopsy.^,

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Important Mitral Regurgitation and Ventricular Dysfunction in Hypereosinophilic Syndrome: A Case Report

Introduction

Cardiac Abnormalities in Hypereosinophilic Syndromes

Abstract

Aortic Valvulitis in Hypereosinophilic Syndrome: A Case Report

Introduction

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Electronic Document Format (Vancouver)

Electronic Document Format (ABNT)

Electronic Document Format (APA)

Electronic Document Format (ISO)

Electronic Document Format (Vancouver)

Electronic Document Format (ABNT)

Electronic Document Format (APA)

Electronic Document Format (ISO)