Pediatric Hypereosinophilic Syndrome Follow-up

Updated: Aug 02, 2018
  • Author: Bruce M Rothschild, MD; Chief Editor: Harumi Jyonouchi, MD  more...
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Follow-up

Further Outpatient Care

Monitoring for eosinophil count, cardiac disease, adverse effects of treatment, and complications is also an essential part of outpatient care.

The frequency of monitoring depends on the apparent stability of disease. Initially, weekly monitoring is indicated; motoring is performed monthly if the patient enters a chronic phase.

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Further Inpatient Care

Monitoring for eosinophil count, cardiac disease, adverse effects of treatment, and complications is essential.

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Inpatient & Outpatient Medications

In the presence of organ involvement, a steroid trial is indicated. If steroidal trial fails, vincristine is used when immediate reduction of eosinophil levels is imperative. Dapsone is considered for skin involvement.

Because most treatment reports are anecdotal, therapy with alkylating agents is the next consideration. Treatment choices should be individualized for each patient.

Because of the risk of thrombotic phenomenon, antiplatelet therapy with aspirin or a NSAID, but not a COX-2–specific agent, is indicated. In the presence of actual thrombotic activity, warfarin (Coumadin) is indicated. Coumadin may also be considered in significant cardiac involvement.

In platelet-derived growth factor receptor alpha (PDGFRA)-associated hypereosinophilic syndrome, imatinib, which inhibits the activity of fusion kinase FIP1L1/PDGFRA, is a first-line treatment.

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Complications

Patients with thrombotic phenomenon require constant monitoring.

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Prognosis

The prognosis is poor, and treatment reports are anecdotal. The mean survival is 9 months. The 3-year survival rate is reported to be 12%. Survival is prolonged if sequelae of organ damage, especially cardiac, can be controlled.

Poor prognostic indicators include the following:

  • Anemia

  • Thrombocytopenia

  • A WBC count higher than 100,000/µL

  • Circulating basophilic abnormal cells

  • Abnormal bone marrow

  • An elevated vitamin B-12 level

  • Abnormal leukocyte alkaline phosphatase levels

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Patient Education

Patients and their families must be alerted to look for signs of thrombotic disease; any change in pulmonary, cardiac, or neurologic status; bruising; or a sore throat. These are indications for urgent reassessment.

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