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Hypereosinophilic Syndrome

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Hypereosinophilic Syndrome

  • See Also
  1. Eosinophilia
  2. Eosinophilia-Myalgia Syndrome
  3. Complete Blood Count
  • Definitions
  1. Hypereosinophilic Syndrome
    1. Group of conditions with Eosinophils >1500 cells/uL >6 months with organ involvement and no cause found
  • Epidemiology
  1. Gender: Men are 10 fold more likely to be affected than women
  2. Age: 20 to 50 years old
  • 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
  • Imaging
  1. Chest XRay
    1. Lung infiltrates seen in >30% of cases
    2. Pleural Effusions
  • Labs
  1. Complete Blood Count (CBC)
    1. Eosinophilia with >=1500 cells/uL (all cases, defining feature)
    2. Thrombocytopenia (>5% of cases)
    3. Anemia
  • 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
  • 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)
  • 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
  • Complications
  1. Angioedema
  2. Arthritis
  3. Congestive Heart Failure, Myocarditis or Restrictive Cardiomyopathy
  4. Deep Vein Thrombosis
  5. Anemia
  6. Hepatomegaly
  7. Splenomegaly
  • Resources
  1. Hypereosinophilic Syndrome (NIH)
    1. https://rarediseases.info.nih.gov/diseases/2804/hypereosinophilic-syndrome
  • References
  1. Mahmoudi (2014) Immunology Made Ridiculously Simple, MedMaster, Miami, FL