Hypereosinophilic syndrome: Difference between revisions
From IDWiki
Content deleted Content added
Imported from text file |
|||
| (2 intermediate revisions by the same user not shown) | |||
| Line 1: | Line 1: | ||
== |
== Background == |
||
* |
*Idiopathic [[eosinophilia]] persisting for more than 6 months without identifiable cause |
||
| ⚫ | |||
| ⚫ | |||
| ⚫ | |||
| ⚫ | |||
==Clinical Manifestations== |
|||
| ⚫ | |||
| ⚫ | |||
| ⚫ | |||
== |
== Differential Diagnosis == |
||
| ⚫ | |||
| ⚫ | |||
| ⚫ | |||
== Investigations == |
|||
* |
*Molecular testing for PDGFRA and FIP1L1 |
||
== |
==Management== |
||
* |
*Steroids |
||
* |
*+/- mepolizumab for steroid-sparing |
||
* |
*+/- imatinib if PDGFRA/FIPL1 positive |
||
== |
==Prognosis== |
||
* |
*Progressive and fatal |
||
[[Category:Immunology]] |
[[Category:Immunology]] |
||
Latest revision as of 17:25, 5 May 2025
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
