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

XII. References

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

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Related Studies

Ontology: Hypereosinophilic syndrome (C1540912)

Definition (MSH) A heterogeneous group of disorders with the common feature of prolonged eosinophilia of unknown cause and associated organ system dysfunction, including the heart, central nervous system, kidneys, lungs, gastrointestinal tract, and skin. There is a massive increase in the number of EOSINOPHILS in the blood, mimicking leukemia, and extensive eosinophilic infiltration of the various organs.
Definition (NCI) A syndrome characterized by persistent eosinophilia, for which no underlying cause can be found, and which is associated with signs of organ involvement and dysfunction. This term has often been applied to cases of chronic eosinophilic leukemia. (WHO, 2001)
Concepts Neoplastic Process (T191)
MSH D017681
SnomedCT 26328002, 393573009, 128835008
English Hypereosinophilic Syndrome, Hypereosinophilic Syndromes, Syndromes, Hypereosinophilic, Syndrome, Hypereosinophilic, hypereosinophilic syndrome (diagnosis), hypereosinophilic syndrome, Hypereosinophilic Syndrome [Disease/Finding], Hypereosinophilic syndrome (disorder), Hypereosinophilic syndrome/chronic eosinophilic leukemia (morphologic abnormality), Hypereosinophilic syndrome (morphologic abnormality), Hypereosinophilic syndrome
Swedish Hypereosinofilt syndrom
Spanish síndrome hipereosinófilo, síndrome hipereosinófilo (anomalía morfológica), Síndrome hipereosinófilo, síndrome hipereosinofílico/leucemia eosinofílica crónica (anomalía morfológica), síndrome hipereosinofílico/leucemia eosinofílica crónica, Síndrome Hipereosinofílico
Japanese コウサンキュウゾウカショウコウグン, 白血病-好酸球性, 特発性好酸球増多症候群, 好酸球増加症候群-特発性, 好酸球増多症候群-特発性, 好酸球性白血病, 特発性好酸球増加症候群, 好酸球増加症候群, 過好酸球症候群
Czech hypereozinofilní syndrom, Hypereozinofilní syndrom
Finnish Hypereosinofiilinen oireyhtymä
Polish Zespół hipereozynofilowy idiopatyczny, Białaczka eozynofilowa, Zapalenie wsierdzia Loefflera, Zespół hipereozynofilowy
Hungarian hypereosinophiliás syndroma
Norwegian Hypereosinofilt syndrom
Portuguese Síndrome hipereosinofílica, Síndrome Hipereosinofílica
Dutch hypereosinofiliesyndroom, Hypereosinofiel syndroom, Syndroom, hypereosinofiel
French Syndrome hyperéosinophilique
German Hypereosinophilie-Syndrom
Italian Sindrome ipereosinofila