Steroid Treatment for Hypereosinophilic Syndrome
Eosinophilia, Hypereosinophilic Syndrome, Leukocyte Disorder
About this trial
This is an interventional treatment trial for Eosinophilia focused on measuring Hypereosinophilia, Hypereosinophilic Syndrome, Corticosteroids, Therapy, HES
Eligibility Criteria
- SUBJECT INCLUSION CRITERIA:
Subjects on Protocol #94-I-0079 will be eligible for participation in the study only if all of the following criteria apply:
- Subjects must be 7 years of age or older to enroll
- Subject meets diagnostic criteria for HES (AEC >1500/microL, absence of a secondary cause and signs and/or symptoms attributable to the eosinophilia)
- AEC greater than 1500 microL obtained within 14 days prior to enrollment
- Willingness to perform the timed steroid challenge
- Appropriate candidate for GC treatment after challenge
- Willingness to have samples stored for future research
SUBJECT EXCLUSION CRITERIA:
A subject will not be eligible to participate in the study if any of the following apply:
- Receiving >10 mg prednisone or equivalent at the time of enrollment.
- Receiving less than or equal to 10 mg of prednisone or equivalent but have not been on a fixed dose for at least 3 weeks (subjects on a current corticosteroid taper will be excluded).
- AEC less than or equal to 1500/microl on the day of the steroid challenge
- Use of immunomodulatory medications, (other than less than or equal to 10 mg/day prednisone) including but not limited to biologics, within the past 6 months.
- Pregnant at the time of screening.
- Have a known mutation in the FIP1L1-PDGFR gene.
- Any condition that, in the opinion of the investigator, places the subject at undue risk by participating in the protocol.
- Weight less than 48 kg (106 lbs) in subjects less than 18 years of age.
Sites / Locations
- National Institutes of Health Clinical Center, 9000 Rockville Pike
Arms of the Study
Arm 1
Other
Steroid Challenge
A single oral dose of prednisone (1 mg/kg rounded to the nearest 5mg) was administered to all participants. The following day, participant began glucocorticoid therapy of prednisone 30 mg oral daily for one week followed by a standardized taper until a minimally effective dose was achieved or participant tapered to prednisone 5 mg oral daily.