Hypereosinophilic Syndrome
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Contents |
Etiology
- unknown
Epidemiology
- rare
- most patients are in the 3rd or 4th decade of life
- male:female ratio is 7:1
Pathology
- absence of obvious reason for eosinophilia
- sustained overproduction of eosinophils in the bone marrow
- tissue infiltration
- end organ damage resulting from eosinophilia
- lung involvement in 40% of patients
- pulmonary infiltrates
- pulmonary fibrosis may develop in chronic cases
- endocardial disease, restrictive cardiomyopathy
Genetics
- fusion of FIP1L1 & PDGFRA ( FIP1L1- PDGFRA), due to an interstitial chromosomal deletion del(4)(q12q12)
Clinical-manifestations
- fever
- night sweats
- weight loss
- cough
- progressive dyspnea
- anorexia
- pruritis
- hepatosplenomegaly
- often asymptomatic
- eosinophilia is often an incidental finding
Laboratory
- complete blood count ( CBC) with differential
- leukocytosis generally > 10,000/ mm3
- eosinophilia > 1500/ mm3 (30-70%) for > 6 months duration
- 10-30% of cases may lack eosinophilia
- peripheral smear:
- normal appearing eosinophils
- no blasts
- bronchoalveolar lavage ( BAL)
- cell count may show > 70% eosinophils
Radiology
Complications
- pulmonary fibrosis may develop in chronic cases
- cardiac complications
- cerebrovascular lesions
- peripheral neuropathy
- often fatal disease
Differential-diagnosis
Management
- supportive
- eosinophilia & clinical course may remain stable for years
- corticosteroids 1st line [2]
- second line therapy
More General Terms
Internet Database
OMIM: 607685
References
- Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 755
- Medical Knowledge Self Assessment Program (MKSAP) 11, 15, American College of Physicians, Philadelphia 1998, 2009
