Hypereosinophilic syndrome: Difference between revisions
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* Mutation in PDGFRA and FIP1L1 in some cases, causes a tyrosine kinase mutation |
* Mutation in PDGFRA and FIP1L1 in some cases, causes a tyrosine kinase mutation |
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== Clinical |
== Clinical Manifestations == |
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* Eosinophilia with cardiomyopathy, skin lesions, thromboembolism, pulmonary disease, neuropathy, hepatosplenomegaly, reduced ventricular size, and eczema |
* Eosinophilia with cardiomyopathy, skin lesions, thromboembolism, pulmonary disease, neuropathy, hepatosplenomegaly, reduced ventricular size, and eczema |
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Revision as of 00:22, 15 July 2020
Definition
- Idiopathic eosinophilia persisting for more than 6 months without identifiable cause
Differential Diagnosis
- See [[../Syndromes/Eosinophilia.md|Eosinophilia]]
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
Investigations
- Molecular testing for PDGFRA and FIP1L1
Management
- Steroids
- +/- mepolizumab for steroid-sparing
- +/- imatinib if PDGFRA/FIPL1 positive
Prognosis
- Progressive and fatal
