Hypereosinophilic syndrome
Unexplained chronic eosinophila / From Wikipedia, the free encyclopedia
Dear Wikiwand AI, let's keep it short by simply answering these key questions:
Can you list the top facts and stats about Endomyocardial fibrosis?
Summarize this article for a 10 year old
Hypereosinophilic syndrome is a disease characterized by a persistently elevated eosinophil count (≥ 1500 eosinophils/mm³) in the blood for at least six months without any recognizable cause, with involvement of either the heart, nervous system, or bone marrow.[5]
Hypereosinophilic syndrome | |
---|---|
Other names | HES.[1] |
Activated eosinophils in the peripheral blood of a patient with idiopathic hypereosinophilic syndrome showing cytoplasmic clearing, nuclear dysplasia, and the presence of immature forms. | |
Specialty | Hematology |
Symptoms | Fatigue, breathlessness, cough, muscle pain, fever, and rash.[2] |
Usual onset | 20-50 years old.[3] |
Types | Primary (or neoplastic) HES, Secondary (or reactive) HES, and Idiopathic HES.[3] |
Diagnostic method | Blood chemistries.[3] |
Differential diagnosis | Acute eosinophilic leukemia, chronic myeloid leukemia, chronic myelomonocytic leukemia, and systemic mastocytosis with eosinophilia.[3] |
Treatment | Corticosteroids, Imatinib, medications to control eosinophil counts, and supportive care.[4] |
Frequency | 0.36 to 6.3 per 100,000.[3] |
Hypereosinophilic syndrome can manifest in many different ways from nonspecific symptoms and fatigue to neurological impairment and endomyocardial fibrosis, which may be fatal.[6]
There are three different variants of hypereosinophilic syndrome, myeloproliferative, lymphocytic, and idiopathic.[7]
HES is a diagnosis of exclusion, after clonal eosinophilia (such as FIP1L1-PDGFRA-fusion induced hypereosinophelia and leukemia) and reactive eosinophilia (in response to infection, autoimmune disease, atopy, hypoadrenalism, tropical eosinophilia, or cancer) have been ruled out.[8][9]
There are some associations with chronic eosinophilic leukemia[10] as it shows similar characteristics and genetic defects.[11] If left untreated, HES is progressive and fatal. It is treated with glucocorticoids such as prednisone.[8] The addition of the monoclonal antibody mepolizumab may reduce the dose of glucocorticoids.[12]