Hypereosinophilic syndrome: Difference between revisions
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== Background == |
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*Idiopathic eosinophilia persisting for more than 6 months without identifiable cause |
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==Clinical Manifestations== |
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== Differential Diagnosis == |
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== Investigations == |
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*Molecular testing for PDGFRA and FIP1L1 |
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==Management== |
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*Steroids |
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*+/- mepolizumab for steroid-sparing |
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*+/- imatinib if PDGFRA/FIPL1 positive |
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==Prognosis== |
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*Progressive and fatal |
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[[Category:Immunology]] |
[[Category:Immunology]] |
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Latest revision as of 14:53, 29 July 2020
Background
- Idiopathic eosinophilia persisting for more than 6 months without identifiable cause
Pathophysiology
- Mutation in PDGFRA and FIP1L1 in some cases, causes a tyrosine kinase mutation
Clinical Manifestations
- Eosinophilia with cardiomyopathy, skin lesions, thromboembolism, pulmonary disease, neuropathy, hepatosplenomegaly, reduced ventricular size, and eczema
Differential Diagnosis
- Refer to Eosinophilia
Diagnosis
- Molecular testing for PDGFRA and FIP1L1
Management
- Steroids
- +/- mepolizumab for steroid-sparing
- +/- imatinib if PDGFRA/FIPL1 positive
Prognosis
- Progressive and fatal
