HYPEREOSINOPHILIC SYNDROME

HYPEREOSINOPHILIC SYNDROME

      Differential History ,Diagnosis ,Pathophysiology ,Treatment Of HYPEREOSINOPHILIC SYNDROME

  • A persistently elevated eosinophil count >1,500 cells/µL for at least 6 months
  • Eosinophil-induced end-organ damage
  • Exclusion of other causes (e.g., parasitic infection, allergy, malignancy, collagen-vascular disease)
  • Almost any organ can be affected, but most patients have bone marrow, cardiac, and central nervous system involvement.
  • It is characterized by more than 1500 eosino­phils/l-JI of peripheral-blood for 6 months or longer.
  • It may also follow lung and bone marrow trans­plantation.
  • Lungs, liver, spleen, skin, nervous system may be affected.
  • Multisystem injury and organ damage caused by excessive numbers of eosinophils in the body.
  • The disease is one of the myelodysplastic disorders.
  • There is dyspnoea, cough, crepts in lungs, of unknown cause.
  • There may be tricuspid valve abnormalities, endomyocardial fibrosis, restrictive cardiomyopa­thy with tissue infiltration by eosinophils.

HYPEREOSINOPHILIC SYNDROME

HYPEREOSINOPHILIC SYNDROME


Pathophysiology HYPEREOSINOPHILIC SYNDROME

  • Organ damage is similar among HES subsets and results from high eosinophil levels.
  • Cytokines IL-3, IL-5, and GM-CSF stimulate bone marrow eosinophil production; IL-5 is most specific.
  • Blood levels, organ migration regulated by chemokines, especially IL-5 and eotaxins
  • HES: Eosinophils infiltrate organs and release toxic granules containing major basic protein, eosinophil peroxidase, eosinophil cationic protein (ECP), eosinophil-derived neurotoxin (EDN), Charcot Leyden crystal, VIP, and substance P. Neurotoxic, cytotoxic, and prothrombotic; creates oxidative burst, reactive oxygen species
  • Cytokine release (IL-1, IL-3, IL-5, TNF-) incites damage and activates inflammatory pathways.
  • EDN and ECP activate fibroblasts: Fibrosis and organ dysfunction

History —

  • Cardiac manifestations (50–60%) may cause heart failure symptoms and chest pain.
  • Cutaneous manifestations (50%) may cause pruritus.
  • Fatigue, anemia
  • GI manifestations (20–30%):
    • Gastritis/enteritis: Diarrhea, vomiting, abdominal pain (embolic bowel infarction)
    • Hepatic manifestations of hepatitis, Budd-Chiari syndrome
  • Left upper quadrant pain (from splenomegaly)
  • Neurologic manifestations (50%) may result from thromboembolic disease: Behavioral changes, memory loss, confusion.
  • Ocular manifestations (20%): Blurry vision/blindness from microemboli
  • Other: Myalgias, arthralgias
  • Pulmonary manifestations (40%) may cause nonproductive cough.

Differential Diagnosis HYPEREOSINOPHILIC SYNDROME

  • Extensive; first rule out secondary causes of eosinophilia: Parasitic infection, allergy, malignancy, drug hypersensitivity, connective-tissue disorders
    • Chronic eosinophilic leukemia: Clonality like F/P+ HES but differs by having 2–20% blasts peripherally or 5–20% blasts in the marrow
    • Acute eosinophilic leukemia: A form of AML with 50–80% eosinophils; may cause bronchospasm, heart failure
  • Other conditions with high eosinophil levels: Hodgkin lymphoma, mastocytosis, chronic myelomonocytic leukemia (eosinophil variant), cutaneous T-cell lymphoma, Churg-Strauss syndrome/other vasculitides, toxicity (the eosinophilia–myalgia syndrome), HIV, HTLV, bronchopulmonary aspergillosis

 

Treatment Of HYPEREOSINOPHILIC SYNDROME

First Line

  • Treatment with corticosteroids orally or IV
  • Dosage and duration are dependent on the patient’s condition.
  • Treatment for 2–4 weeks beyond clinical improvement has been suggested.
  • Discontinuing offending drug.
  • Treatment of underlying parasite infestation

Second Line

  • Some patients may require oxygen therapy.

Some Spatial —

  • Glucocorticoids Hydroxyurea
  • Anticoagulants for patients with thromboembolic complication; corticosteroids.
  • Patients unresponsive to corticosteroids have shown marked improvement when given cytotoxic agents such as hydroxyurea
  • Treatment of associated conditions.