idiopathic hypereosinophilic syndrome
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2021 ◽  
Vol 5 (02) ◽  
Author(s):  
Mahbuba Sharmin ◽  
Amin Lutful Kabir ◽  
Md. Abdul Aziz ◽  
Sonia Shormin Miah ◽  
Umme Shahera ◽  
...  

World Health Organization defines a rare diagnosis Idiopathic hypereosinophilic syndrome (HES) as a persistent eosinophilia for 6 months and resulting in end-organ dysfunction. Most of the patients present with nonspecific symptoms, while others will present with symptoms of the affected organs, commonly those involving the heart, skin, or nervous system. Gastrointestinal or liver involvement is estimated to affect up to one-third of patients with HES, although patients with clinically significant disease are limited to case reports. This is the first report of a patient presenting with idiopathic HES related hepatitis and achalasia. Hypereosinophilic syndrome has been reported to be associated with hepatic dysfunction; liver histology is mainly characterized by a diffuse eosinophilic inflammatory infiltrate. A 49-yr-old woman, diagnosed as a case of idiopathic hypereosinophilic syndrome with bone marrow and pulmonary eosinophilic infiltrates associated with peripheral eosinophilia,high IgE level  developed features of chronic gatrornteritis, hepatitis, with a significant eosinophil component. She responded well to systemic glucocorticoid  and Imatinib therapy with normalization of liver function tests within a few weeks.


Author(s):  
Esra Turğut ◽  
Murat Alpua ◽  
Selim Yalçın ◽  
Oya Coşkun ◽  
Mirace Yasemin Karadeniz Bilgili ◽  
...  

Cureus ◽  
2021 ◽  
Author(s):  
Magda S Silva ◽  
Carina Ramalho ◽  
Francelino Ferreira ◽  
Inês Maia ◽  
Anneke Joosten

Imaging ◽  
2021 ◽  
Author(s):  
Laura Ceriello ◽  
Antonino Scarinci ◽  
Cesare Mantini ◽  
Sabina Gallina ◽  
Filippo Cademartiri ◽  
...  

Abstract A 27-years-old female with multiple autoimmune disorders presented to our cardiology unit for acute chest pain and worsening dyspnoea. Admission blood tests revealed increased serum levels of high-sensitive cardiac troponin, eosinophilic count and C-reactive protein. Laboratory findings, low QRS voltages by ECG, mildly reduced left ventricular systolic function in the context of pseudohypertrophy, mild and diffuse late gadolinium enhancement associated with markedly increased native T1 and T2 mapping levels assessed by echocardiography and cardiovascular magnetic resonance imaging, raised the suspicion of massive eosinophilic myocarditis, subsequently confirmed by histological examination of endomyocardial biopsy. Prompt initiation of immunosuppressive treatment allowed swift regression of myocardial inflammation and full recovery of left ventricular systolic function within one month. After ruling-out clonal myeloid disorder, lymphocyte-variant and reactive hypereosinophilia, the young lady was eventually diagnosed with idiopathic hypereosinophilic syndrome. This case report turns the spotlight on the role and importance of advanced multi-modality cardiovascular imaging for raising clinical suspicion of acute eosinophilic myocarditis, guiding diagnostic work-up and monitoring response to treatment.


Author(s):  
Inês Rueff Rato ◽  
Joana Rigor ◽  
Paula Ferreira ◽  
Joana Laranjinha ◽  
Gualter Santos Silva ◽  
...  

Hypereosinophilic syndrome (HES) is a heterogenous group of diseases characterized by abnormal accumulation of eosinophils in the blood or peripheral tissues. It can affect all organs and therefore clinical manifestations are highly variable. We describe the case of a 38-year-old man admitted for febrile polyserositis. He developed cardiac tamponade requiring pericardiocentesis complicated by left ventricle perforation which was successfully repaired. He presented mild peripheral eosinophilia. Bronchoalveolar lavage evidenced eosinophilic alveolitis, and pleural and pericardium histopathology revealed the presence of abundant eosinophils. All other causes of tissue eosinophilia were excluded and the diagnosis of idiopathic HES was made. The patient was started on glucocorticoids with resolution of symptoms. This case report describes a rare but potentially fatal presentation of HES and demonstrates the difficulty and delay in diagnosis when peripheral hypereosinophilia is absent.


Author(s):  
D. Saadouli ◽  
K. Ben Mansour ◽  
S. Sallem ◽  
L. Ammari ◽  
M.A. El Afrit ◽  
...  

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