Idiopathic Hypereosinophilic Syndrome with Multiple Organ Involvement

Abstract

Idiopathic hypereosinophilic syndrome is a rare disease which is diagnosed after excluding other conditions. The syndrome is characterized by multiple organ involvement including the heart, nervous system, lungs, and gastrointestinal tract. The disease is suspected if there is peripheral blood eosinophilia and no clear etiology. The main treatment is corticosteroids. Patients who do not respond to corticosteroids can be treated with imatinib, immunomodulatory agents, myelosuppressive therapy, or mepolizumab. Alemtuzumab can be considered in severe cases that are unresponsive to other therapies. In this paper, we describe a case of idiopathic hypereosinophilic syndrome with mainly cardiac system involvement and left ventricular thrombus formation which was complicated by cerebral thromboemboli while on warfarin with international normalized ratio in the therapeutic range. Our patient responded well to steroids appreciated by improvement in clinical symptoms and decrease in eosinophil count.

A Diagnosis of Idiopathic Hypereosinophilic Syndrome With Skin Involvement in a Patient Presenting With Lymphadenopathy and Rash

“…There would be a drastic amelioration in the blood eosinophilia and therefore paves a way to assess the response. Mostly, the HIS cases are described all around the world with mainly cardiac and gastrointestinal involvement [ 1 ]. In addition, there are some case reports of patients with HES presenting with embolic stroke [ 7 ].…”

“…HES is categorized into three main groups according to the etiology. They are primary, secondary, and idiopathic HES [ 1 ]. When the etiological workup excludes any causative agent, it falls under the idiopathic HES group.…”

Case report: Application of non-VKA oral anticoagulants in patient of idiopathic hypereosinophilic syndrome with intracardiac thrombus

“…Idiopathic hypereosinophilic syndrome (IHES) is a myeloproliferative disorder characterized by persistent eosinophilia. The occurrence of this disease is rare, but it often causes serious damage to multiple organs and has high mortality ( He et al, 2020 ; Abo Shdid et al, 2021 ; Wang et al, 2021 ). It was initially documented by Anderson and Hardy in 1968 ( Anderson and Hardy, 1968 ), while the diagnosis of IHES was first defined by Chusid et al (1975) .…”

“…It was initially documented by Anderson and Hardy in 1968 ( Anderson and Hardy, 1968 ), while the diagnosis of IHES was first defined by Chusid et al (1975) . The diagnosis of this disease usually includes the following criteria ( Abo Shdid et al, 2021 ): 1) the peripheral eosinophil count is more than 1.5 × 10^9/L for more than 6 months; 2) other diseases leading to increased eosinophils, such as parasitic diseases, allergic diseases, drug allergies, lymphoma, HIV infection, and nonhematologic malignancies, are excluded; and 3) there are clear symptoms and signs of organ dysfunction due to eosinophil infiltration, such as hepatosplenomegaly, heart failure, heart murmur, diffuse and local neurological sign abnormalities, pulmonary fibrosis, fever, weight loss and anemia. Multiorgan involvement is frequently noted in these patients.…”

Resolved Hypereosinophilic Syndrome and Immune Thrombocytopenic Purpura in Ulcerative Colitis Patients Post Colectomy: A Case Series and Literature Review

“…20 Several pathogenic mechanisms have been proposed in HES, ie, overproduction of eosinophilopoietic cytokines, promoting the eosinophil activity, and defective physiological suppression of eosinophils. 20,21 Eosinophils production from the bone marrow is regulated by specific cytokines, ie, IL-3, IL-5, and granulocyte-macrophage colony-stimulating factor (GM-CSF). 20 Overproduction of eosinophils in HES is proposed to be either due to the primary involvement of myeloid cells (ie, creation of the FIP1L1-PDGFRA fusion gene) or excessive production of IL-5 from clonally expanded T cells.…”