Hypereosinophilic syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and keywords: HES; Hypereosinophilic disease;

Overview

Hypereosinophilic syndrome (HES) is a disease characterized by a persistently elevated eosinophil count (≥ 1500 eosinophils/mm³) in the blood for at least six months without any recognizable cause, with involvement of either the heart, nervous system, or bone marrow. Hypereosinophilic syndrome is a diagnosis of exclusion, after clonal eosinophilia (such as leukemia) and reactive eosinophilia (in response to infection, autoimmune disease, atopy, hypoadrenalism, tropical eosinophilia, or cancer) have been ruled out. There are some associations with chronic eosinophilic leukemia as it shows similar characteristics and genetic defects. If left untreated, hypereosinophilic syndrome is progressively fatal. It is treated with glucocorticoids such as prednisone. The addition of the monoclonal antibody mepolizumab may reduce the dose of glucocorticoids.

Historical Perspective

• Hypereosinophilic syndrome was first described in 1968.
• In 2003, PDGFRA and FIP1L1 genes mutations were first identified in the pathogenesis of hypereosinophilic syndrome.

Classification

• Hypereosinophilic syndrome may be classified into 2 groups:

• Endomyocardial fibrosis

• Also known as Davies disease

• Loeffler's endocarditis

• Other variants of hypereosinophilic syndrome may include myeloproliferative, T lymphocytic, familiar, idiopathic, and organ-restricted hypereosinophilic syndrome variant.

Pathophysiology

• The pathogenesis of hypereosinophilic syndrome is characterized by [feature1], [feature2], and [feature3].
• Genes associated with the development of hypereosinophilic syndrome,include:

• PDGFRA gene
• PDGFRB gene
• FGFR1 gene

• On gross pathology,there are no are characteristic findings of hypereosinophilic syndrome.
• On microscopic histopathological analysis, hypercellular marrow, and increased eosinophilic precursors are characteristic findings of hypereosinophilic syndrome.

Causes

• Hypereosinophilic syndrome may be caused by either [cause1], [cause2], or [cause3].
• Hypereosinophilic syndrome is caused by a mutation in the BCR-ABL, PDGFRA, PDGFRβ, and KIT gene.

Differentiating Hypereosinophilic Syndrome from Other Diseases

• Hypereosinophilic syndrome must be differentiated from other diseases that cause skin rash, fatigue, and hypereosinophilia, such as:

• Allergic diseases
• Atopic dermatitis
• Drug reactions
• Eosinophilic pneumonia
• Hypersensitivity diseases
• Malignancy with secondary eosinophilia

Epidemiology and Demographics

• Hypereosinophilic syndrome is a very rare disease.
• The prevalence of hypereosinophilic syndrome is approximately 0.36 to 6.3 per 100,000 individuals worldwide.

Age

• Patients of all age groups may develop hypereosinophilic syndrome.
• Hypereosinophilic syndrome is more commonly observed among adults.

Gender

• Males are more likely to be affected from hypereosinophilic syndrome than females, with male to female ratio of 5:1

Race

• Caucasian individuals are more likely to develop cancer of unknown primary origin.

Risk Factors

• Common risk factors in the development of hypereosinophilic syndrome are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

Natural History, Complications and Prognosis

• The majority of patients with hypereosinophilic syndrome remain asymptomatic for [duration/years].
• Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
• If left untreated, [#%] of patients with hypereosinophilic syndrome may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
• Common complications of hypereosinophilic syndrome include [complication 1], [complication 2], and [complication 3].
• Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with hypereosinophilic syndrome is approximately [#%]

Diagnosis

Diagnostic Criteria

• The diagnosis of hypereosinophilic syndrome includes the following findings:

• Persistently elevated eosinophil count (≥ 1500 eosinophils/mm³) in the blood

• At least six months without any recognizable cause
• Involvement of either the heart, nervous system, or bone marrow.

Symptoms

• Symptoms of hypereosinophilic syndrome may include the following:

• Shortness of breath
• Skin rash
• Cough
• Diarrhea
• Myalgias
• Fatigue
• Weight-loss

Physical Examination

• Patients with hypereosinophilic syndrome usually have a normal appearance.
• Physical examination may be remarkable for:

• Skin rash

• Thickening of the skin (lichenification)
• Dermographism

• Low-grade fever
• Raynaud phenomenon
• Wheezing

Laboratory Findings

• Laboratory findings consistent with the diagnosis of hypereosinophilic syndrome include the following:

• Elevated serum IgE immunoglobulin
• Elevated serum vitamin B12
• Elevated serum tryptase

Imaging Findings

• There are no imaging findings associated with hypereosinophilic syndrome.

Other Diagnostic Studies

• Hypereosinophilic syndrome may also be diagnosed using [diagnostic study name].
• Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

• The mainstay of therapy for hypereosinophilic syndrome, include the following:

Surgery

• Surgical intervention is not recommended for the management of hypereosinophilic syndrome.

Prevention

• There are no primary preventive measures available for hypereosinophilic syndrome.
• There are no effective measures for the primary prevention of hypereosinophilic syndrome.
• Once diagnosed and successfully treated, patients with hypereosinophilic syndrome are followed-up every 12 months.
• Follow-up testing includes the following tests: