Hypereosinophilic Syndrome

Aka: Hypereosinophilic Syndrome

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II. Definitions

  1. Hypereosinophilic Syndrome
    1. Group of conditions with Eosinophils >1500 cells/uL >6 months with organ involvement and no cause found

III. Epidemiology

  1. Gender: Men are 10 fold more likely to be affected than women
  2. Age: 20 to 50 years old

IV. Symptoms

  1. Symptoms vary by specific Hypereosinophilic Syndrome
  2. Fever
  3. Cardiopulmonary
    1. Angioedema
    2. Cough
    3. Shortness of Breath
    4. Chest Pain
  4. Rheumatologic
    1. Myalgias
    2. Arthralgias
  5. Neurologic
    1. Vertigo
    2. Weakness
    3. Blurred Vision
  6. Skin
    1. Rash
    2. Pruritus

V. Imaging

  1. Chest XRay
    1. Lung infiltrates seen in >30% of cases
    2. Pleural Effusions

VI. Labs

  1. Complete Blood Count (CBC)
    1. Eosinophilia with >=1500 cells/uL (all cases, defining feature)
    2. Thrombocytopenia (>5% of cases)
    3. Anemia

VII. Diagnosis

  1. Absolute Eosinophil Count >=1500 cells/uL for 6 months or more AND
  2. No cause identified AND
  3. Organ involvement with Eosinophilic tissue infiltration

VIII. Differential Diagnosis

  1. See Eosinophilia
  2. Familial Hypereosinophilic Syndrome (rare)
  3. Myeloproliferative Hypereosinophilic Syndrome (e.g. myeloid Leukemia, See Eosinophilia)
  4. Lymphocytic Hypereosinophilic Syndrome
    1. Increased Interkeukin-5 (Cytokine)

IX. Management

  1. First-Line Management
    1. Corticosteroids (e.g. Prednisone)
  2. Second-Line Management Options (Corticosteroid refractory Hypereosinophilia)
    1. Hydroxyurea
    2. Interferon-Alpha
    3. Imatinab (Gleevec)
  3. Third-Line Refractory Cases
    1. Cyclosporin
    2. Bone Marrow Transplant
  4. Other Specific Management
    1. Myeloproliferative Hypereosinophilic Syndrome
      1. Imatinab (Gleevec) is indicated in FIP1L1/PDGFRA Fusion Gene

X. Complications

  1. Angioedema
  2. Arthritis
  3. Congestive Heart Failure, Myocarditis or Restrictive Cardiomyopathy
  4. Deep Vein Thrombosis
  5. Anemia
  6. Hepatomegaly
  7. Splenomegaly

XI. Resources

  1. Hypereosinophilic Syndrome (NIH)
    1. https://rarediseases.info.nih.gov/diseases/2804/hypereosinophilic-syndrome

XII. References

  1. Mahmoudi (2014) Immunology Made Ridiculously Simple, MedMaster, Miami, FL

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