Idiopathic hypereosinophilic syndrome presenting with multiple organ damage

Abstract The idiopathic hypereosinophilic syndrome, a disorder characterized by peripheral blood and bone marrow eosinophilia associated with single or multiple organ system dysfunction attributable to tissue invasion by eosinophils has, in the past, been associated with an extremely poor prognosis. Recently, we reported the favorable impact of a therapeutic protocol consisting of prednisone and/or hydroxyurea on the morbidity and mortality of this syndrome. We have reviewed the clinical and hematologic features upon admission and the subsequent clinical courses of 32 patients with this disease referred to the NIH between 1965 and 1979 in an effort to determine which features suggest a more rapidly progressive course. A grading system based on 22 clinical features involving the 8 organ systems commonly affected by the illness was devised. The disease followed a more aggressive course in patients with evidence of cardiac or neurologic dysfunction at the time of initial NIH evaluation. Although splenomegaly, in and of itself, caused little morbidity, splenic enlargement at presentation appeared to be a predictor of a more aggressive course. The clinical grading system accurately predicted which patients would require no specific antihypereosinophilic therapy, which patients would respond adequately to corticosteroids, and which patients would require therapy with cytotoxic agents. It is proposed that this clinical grading system, and the hematologic grading system outlined in the accompanying report be used as aids in the selection of initial therapy in this group of patients.

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A clinicopathologic correlation of the idiopathic hypereosinophilic syndrome. II. Clinical manifestations

Blood ◽

10.1182/blood.v58.5.1021.bloodjournal5851021 ◽

1981 ◽

Vol 58 (5) ◽

pp. 1021-1026 ◽

Cited By ~ 3

Author(s):

RT Schooley ◽

MA Flaum ◽

HR Gralnick ◽

AS Fauci

Keyword(s):

Hypereosinophilic Syndrome ◽

Clinical Manifestations ◽

Initial Therapy ◽

Multiple Organ ◽

Cytotoxic Agents ◽

Grading System ◽

Idiopathic Hypereosinophilic Syndrome ◽

Splenic Enlargement ◽

Organ Systems ◽

Clinical Grading

The idiopathic hypereosinophilic syndrome, a disorder characterized by peripheral blood and bone marrow eosinophilia associated with single or multiple organ system dysfunction attributable to tissue invasion by eosinophils has, in the past, been associated with an extremely poor prognosis. Recently, we reported the favorable impact of a therapeutic protocol consisting of prednisone and/or hydroxyurea on the morbidity and mortality of this syndrome. We have reviewed the clinical and hematologic features upon admission and the subsequent clinical courses of 32 patients with this disease referred to the NIH between 1965 and 1979 in an effort to determine which features suggest a more rapidly progressive course. A grading system based on 22 clinical features involving the 8 organ systems commonly affected by the illness was devised. The disease followed a more aggressive course in patients with evidence of cardiac or neurologic dysfunction at the time of initial NIH evaluation. Although splenomegaly, in and of itself, caused little morbidity, splenic enlargement at presentation appeared to be a predictor of a more aggressive course. The clinical grading system accurately predicted which patients would require no specific antihypereosinophilic therapy, which patients would respond adequately to corticosteroids, and which patients would require therapy with cytotoxic agents. It is proposed that this clinical grading system, and the hematologic grading system outlined in the accompanying report be used as aids in the selection of initial therapy in this group of patients.

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Idiopathic Hypereosinophilic Syndrome with Multiple Organ Involvement

Case Reports in Oncology ◽

10.1159/000511396 ◽

2021 ◽

pp. 249-255

Author(s):

Reham Abo Shdid ◽

Bahjat Azrieh ◽

Seham Alebbi ◽

Shafik Mansour ◽

Muhammad Naeem

Keyword(s):

Clinical Symptoms ◽

Thrombus Formation ◽

Hypereosinophilic Syndrome ◽

International Normalized Ratio ◽

Multiple Organ ◽

Left Ventricular ◽

Organ Involvement ◽

Idiopathic Hypereosinophilic Syndrome ◽

Immunomodulatory Agents ◽

Cardiac System

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.

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Idiopathic hypereosinophilic syndrome with multiple organ infarction: improvement of the skin condition masking the course of disease

European Journal of Dermatology ◽

10.1684/ejd.2014.2307 ◽

2014 ◽

Vol 24 (2) ◽

pp. 274-275

Author(s):

Hannes TRATTNER ◽

Friedrich BREIER ◽

Andreas STEINER ◽

Marlies WRUHS ◽

Robert FELDMANN

Keyword(s):

Hypereosinophilic Syndrome ◽

Skin Condition ◽

Multiple Organ ◽

Idiopathic Hypereosinophilic Syndrome ◽

Course Of Disease

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Efficacy of Imatinib Mesylate in a Patient with Idiopathic Hypereosinophilic Syndrome and Severe Heart Involvement

Tumori Journal ◽

10.1177/030089160509100112 ◽

2005 ◽

Vol 91 (1) ◽

pp. 67-70 ◽

Cited By ~ 6

Author(s):

Gabriel Anghel ◽

Luca De Rosa ◽

Carla Ruscio ◽

Nicola Petti ◽

Massimo Riccardi ◽

...

Keyword(s):

Imatinib Mesylate ◽

Hypereosinophilic Syndrome ◽

Young Male ◽

Organ Damage ◽

Imatinib Treatment ◽

Idiopathic Hypereosinophilic Syndrome ◽

Hematological Disease ◽

Heart Involvement ◽

Unsuccessful Treatment ◽

History Of

Idiopathic hypereosinophilic syndrome (HES) is a rare, chronic hematological disease mainly characterized by unexplained prolonged eosinophilia, with frequent evidence of secondary organ damage. Treatment with steroids, chemotherapy, interferon-alpha (IFN-α), or imatinib-mesylate may improve the prognosis. Here we describe the case of a young male patient with a six-year history of HES and severe heart involvement who, after unsuccessful treatment attempts with steroids, hydroxyurea and IFN-α, had a prompt, clinical and hematological complete remission following administration of imatinib. As his cardiac function also markedly improved, he was considered for heart transplant. However, seven years after the onset of the disease and four months after the termination of imatinib treatment the patient died of a cerebral hemorrhage that occurred during an episode of acute respiratory sepsis. Imatinib has been previously reported to be effective in some hematological conditions with no evidence of the BCR/ABL transcript. The mechanisms that are probably involved in the response to imatinib in HES are also discussed.

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A Case Report of Eosinophilic Esophagitis Accompanying Hypereosinophilic Syndrome

Case Reports in Gastrointestinal Medicine ◽

10.1155/2012/683572 ◽

2012 ◽

Vol 2012 ◽

pp. 1-3

Author(s):

Mahreema Jawairia ◽

Ghulamullah Shahzad ◽

Jaspreet Singh ◽

Kaleem Rizvon ◽

Paul Mustacchia

Keyword(s):

Bone Marrow ◽

Hypereosinophilic Syndrome ◽

Organ Damage ◽

Multiple Organ ◽

Peripheral Eosinophilia ◽

Gi Tract ◽

Blood Disorder ◽

Organ Systems ◽

History Of ◽

Tract Involvement

Hypereosinophilic syndrome is a blood disorder characterized by the overproduction of eosinophils in the bone marrow with persistent peripheral eosinophilia, associated with organ damage by the release of eosinophilic mediators. Although HES can involve multiple organ systems, GI tract involvement is very rare. Few cases of HES presenting with gastritis or enteritis have been reported worldwide. To date, HES presenting with esophagus involvement has only been reported once. Here, we present a 39-year-old Hispanic female patient with history of HES presenting with complaints of dysphagia and generalized pruritus.

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