Hypereosinophilic syndrome

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Activated Eosinophils in Idiopathic Hypereosinophilic Syndrome (9125007255)

Hypereosinophilic syndrome (HES) is a group of rare blood disorders characterized by the overproduction of eosinophils, a type of white blood cell. This condition can lead to damage in various organs and tissues due to the accumulation of eosinophils. HES is typically diagnosed when a patient has a persistently high eosinophil count (greater than 1,500 eosinophils per microliter of blood) for more than six months without a known cause.

Classification[edit | edit source]

HES can be classified into several subtypes based on the underlying cause and clinical presentation:

  • Myeloproliferative HES - associated with genetic mutations such as the FIP1L1-PDGFRA fusion gene.
  • Lymphocytic HES - associated with abnormal T cells that produce eosinophil-stimulating factors.
  • Idiopathic HES - where no specific cause can be identified.

Symptoms[edit | edit source]

The symptoms of HES can vary widely depending on the organs affected. Common symptoms include:

Diagnosis[edit | edit source]

The diagnosis of HES involves several steps:

  • Complete blood count (CBC) to measure eosinophil levels.
  • Bone marrow biopsy to examine the production of eosinophils.
  • Genetic testing to identify mutations such as the FIP1L1-PDGFRA fusion gene.
  • Imaging studies (e.g., CT scan, MRI) to assess organ involvement.

Treatment[edit | edit source]

Treatment for HES aims to reduce eosinophil levels and prevent organ damage. Common treatments include:

Prognosis[edit | edit source]

The prognosis for HES varies depending on the subtype and response to treatment. Early diagnosis and appropriate therapy can improve outcomes and reduce the risk of complications.

Related Pages[edit | edit source]

Categories[edit | edit source]


Contributors: Prab R. Tumpati, MD