Unifying Concepts

Diagnostic Criteria for Idiopathic Hypereosinophilic Syndrome (HES)

Diagnostic Criteria for Idiopathic Hypereosinophilic Syndrome (HES) are:

1. Persistent eosinophilia of over 1500/cubic millimeter for longer than 6 month;

2. Lack of evidence of other known causes of secondary hypereosinophilia (SH);

3. Multiple organ involvement.

Diseases Associated with Eosinophilia

“Allergic” Diseases

  • Atopic and related diseases
  • Medication-related eosinophilias

Infectious Diseases

  • Parasitic infections, mostly with helminths
  • Specific fungal infections: allergic bronchopulmonary aspergillosis, coccidioidomycosis (acute and sometimes disseminated)
  • Other infections–infrequent, including HIV-1 and HTLV-1

Hematologic and Neoplastic Disorders

  • Hypereosinophilic syndrome
  • Leukemia
  • Lymphomas, including nodular sclerosing Hodgkin’s disease
  • Tumor associated
  • Mastocytosis

Diseases with Specific Organ Involvement

  • Skin and subcutaneous diseases, including urticaria, bullous pemphigoid, eosinophilic cellulitis (Well’s syndrome), episodic angioedema with eosinophilia
  • Pulmonary diseases, including acute or chronic eosinophilic pneumonia, allergic bronchopulmonary aspergillosis
  • Gastrointestinal diseases, including eosinophilic gastroenteritis
  • Neurologic diseases (e.g., eosinophilic meningitis)
  • Rheumatologic diseases, especially Churg-Strauss vasculitis; also eosinophilic fasciitis
  • Cardiac diseases (e.g., endomyocardial fibrosis)
  • Renal diseases, including drug-induced interstitial nephritis, eosinophilic cystitis, dialysis

Immunologic Reactions

  • Specific immune deficiency diseases: hyper-IgE syndrome, Omenn’s syndrome
  • Transplant rejection: lung, kidney, liver


  • Hypoadrenalism: Addison’s disease, adrenal hemorrhage


  • Atheroembolic disease
  • Irritation of serosal surfaces, including peritoneal dialysis
  • Inherited


  1. Roufosse F, Cogan E, Goldman M. The hypereosinophilic syndrome revisited. Annu Rev Med. 2003;54:169-84. Epub 2001 Dec 3. [Medline]


Created: Jun 21, 2005

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