scholarly journals Investigating Febrile Polyserositis: An Unusual Case of Idiopathic Hypereosinophilic Syndrome

2021 ◽  
Author(s):  
Inês Rueff Rato ◽  
Joana Rigor ◽  
Paula Ferreira ◽  
Joana Laranjinha ◽  
Gualter Santos Silva ◽  
...  
Keyword(s):  
Unusual Case ◽  
Hypereosinophilic Syndrome ◽  
Clinical Manifestations ◽  
Idiopathic Hypereosinophilic Syndrome ◽  
Peripheral Tissues ◽  
Delay In Diagnosis ◽  
Tissue Eosinophilia ◽  
Heterogenous Group ◽  
Abnormal Accumulation ◽  
Eosinophilic Alveolitis

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.

A clinicopathologic correlation of the idiopathic hypereosinophilic syndrome. II. Clinical manifestations

Blood ◽  
1981 ◽  
Vol 58 (5) ◽  
pp. 1021-1026 ◽  
Author(s):  
RT Schooley ◽  
MA Flaum ◽  
HR Gralnick ◽  
AS Fauci
Keyword(s):  
Hypereosinophilic Syndrome ◽  
Clinical Manifestations ◽  
Initial Therapy ◽  
Multiple Organ ◽  
Cytotoxic Agents ◽  
Grading System ◽  
Idiopathic Hypereosinophilic Syndrome ◽  
Splenic Enlargement ◽  
Organ Systems ◽  
Clinical Grading

Abstract The idiopathic hypereosinophilic syndrome, a disorder characterized by peripheral blood and bone marrow eosinophilia associated with single or multiple organ system dysfunction attributable to tissue invasion by eosinophils has, in the past, been associated with an extremely poor prognosis. Recently, we reported the favorable impact of a therapeutic protocol consisting of prednisone and/or hydroxyurea on the morbidity and mortality of this syndrome. We have reviewed the clinical and hematologic features upon admission and the subsequent clinical courses of 32 patients with this disease referred to the NIH between 1965 and 1979 in an effort to determine which features suggest a more rapidly progressive course. A grading system based on 22 clinical features involving the 8 organ systems commonly affected by the illness was devised. The disease followed a more aggressive course in patients with evidence of cardiac or neurologic dysfunction at the time of initial NIH evaluation. Although splenomegaly, in and of itself, caused little morbidity, splenic enlargement at presentation appeared to be a predictor of a more aggressive course. The clinical grading system accurately predicted which patients would require no specific antihypereosinophilic therapy, which patients would respond adequately to corticosteroids, and which patients would require therapy with cytotoxic agents. It is proposed that this clinical grading system, and the hematologic grading system outlined in the accompanying report be used as aids in the selection of initial therapy in this group of patients.

Response to Vincristine Treatment in a Case of Idiopathic Hypereosinophilic Syndrome with Multiple Clinical Manifestations

1984 ◽  
Vol 72 (1) ◽  
pp. 21-25 ◽  
Author(s):  
E. Cofrancesco ◽  
M. Cortellaro ◽  
E. Pogliani ◽  
C. Boschetti ◽  
M. Salvatore ◽  
...  
Keyword(s):  
Hypereosinophilic Syndrome ◽  
Clinical Manifestations ◽  
Idiopathic Hypereosinophilic Syndrome

Hepatitis in Idiopathic Hypereosinophilic Syndrome: Report of an Unusual Case

2021 ◽  
Vol 5 (02) ◽  
Author(s):  
Mahbuba Sharmin ◽  
Amin Lutful Kabir ◽  
Md. Abdul Aziz ◽  
Sonia Shormin Miah ◽  
Umme Shahera ◽  
...  
Keyword(s):  
Unusual Case ◽  
Case Reports ◽  
Hypereosinophilic Syndrome ◽  
Liver Function Tests ◽  
World Health ◽  
Idiopathic Hypereosinophilic Syndrome ◽  
Clinically Significant ◽  
Health Organization ◽  
Significant Disease ◽  
Rare Diagnosis

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.

Diversity of clinical manifestations and response to corticosteroids for idiopathic hypereosinophilic syndrome: retrospective study in 33 patients

2012 ◽  
Vol 54 (4) ◽  
pp. 807-811 ◽  
Author(s):  
Grzegorz Helbig ◽  
Katarzyna Wiśniewska-Piąty ◽  
Tomasz Francuz ◽  
Joanna Dziaczkowska-Suszek ◽  
Sławomira Kyrcz-Krzemień
Keyword(s):  
Retrospective Study ◽  
Hypereosinophilic Syndrome ◽  
Clinical Manifestations ◽  
Idiopathic Hypereosinophilic Syndrome

scholarly journals A clinicopathologic correlation of the idiopathic hypereosinophilic syndrome. II. Clinical manifestations

Blood ◽  
1981 ◽  
Vol 58 (5) ◽  
pp. 1021-1026 ◽  
Author(s):  
RT Schooley ◽  
MA Flaum ◽  
HR Gralnick ◽  
AS Fauci
Keyword(s):  
Hypereosinophilic Syndrome ◽  
Clinical Manifestations ◽  
Initial Therapy ◽  
Multiple Organ ◽  
Cytotoxic Agents ◽  
Grading System ◽  
Idiopathic Hypereosinophilic Syndrome ◽  
Splenic Enlargement ◽  
Organ Systems ◽  
Clinical Grading

The idiopathic hypereosinophilic syndrome, a disorder characterized by peripheral blood and bone marrow eosinophilia associated with single or multiple organ system dysfunction attributable to tissue invasion by eosinophils has, in the past, been associated with an extremely poor prognosis. Recently, we reported the favorable impact of a therapeutic protocol consisting of prednisone and/or hydroxyurea on the morbidity and mortality of this syndrome. We have reviewed the clinical and hematologic features upon admission and the subsequent clinical courses of 32 patients with this disease referred to the NIH between 1965 and 1979 in an effort to determine which features suggest a more rapidly progressive course. A grading system based on 22 clinical features involving the 8 organ systems commonly affected by the illness was devised. The disease followed a more aggressive course in patients with evidence of cardiac or neurologic dysfunction at the time of initial NIH evaluation. Although splenomegaly, in and of itself, caused little morbidity, splenic enlargement at presentation appeared to be a predictor of a more aggressive course. The clinical grading system accurately predicted which patients would require no specific antihypereosinophilic therapy, which patients would respond adequately to corticosteroids, and which patients would require therapy with cytotoxic agents. It is proposed that this clinical grading system, and the hematologic grading system outlined in the accompanying report be used as aids in the selection of initial therapy in this group of patients.

VP-011 AN UNUSUAL CASE OF IDIOPATHIC HYPEREOSINOPHILIC SYNDROME PRESENTING WITH MYOPERICARDITIS AND A POLIPOID CARDIAC MASS

2012 ◽  
Vol 155 ◽  
pp. S94-S95
Author(s):  
Ö.U. Elalmiş ◽  
H. Çiçekçioğlu ◽  
C. Mansuroğlu ◽  
M. Şahin ◽  
G. Ergün ◽  
...  
Keyword(s):  
Unusual Case ◽  
Hypereosinophilic Syndrome ◽  
Cardiac Mass ◽  
Idiopathic Hypereosinophilic Syndrome

scholarly journals Idiopathic hypereosinophilic syndrome with pulmonary hypertension

2018 ◽  
Vol 9 (1) ◽  
pp. 204589401879399
Author(s):  
Liping Zhang ◽  
Xia Peng ◽  
Binay Kumar Adhikari ◽  
Bo Li ◽  
Quan Liu ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Right Ventricular ◽  
Hypereosinophilic Syndrome ◽  
Systolic Pressure ◽  
Clinical Manifestations ◽  
Lower Extremities ◽  
Pulmonary Artery Systolic Pressure ◽  
Heart Catheterization ◽  
Idiopathic Hypereosinophilic Syndrome

Hypereosinophilic syndrome is a myeloproliferative disorder characterized by persistent eosinophilia with involvement of multiple organs. The occurrence of severe pulmonary hypertension (PH) in the setting of hypereosinophilic syndrome is very uncommon. A 43-year-old man with documented idiopathic hypereosinophlic syndrome presented to the hospital with symptoms of paroxysmal chest discomfort and progressive exertional dyspnea. Physical examination showed distended jugular veins, cyanosed lips, increased P2 sound, and moderate pitting edema of the lower extremities. Echocardiography revealed enlarged right atrium, enlarged right ventricle, increased pulmonary artery systolic pressure, and decreased right ventricular systolic function. Venous ultrasound of the lower extremities, computed tomography pulmonary angiography, and right heart catheterization (RHC) were negative for thrombus. The pulmonary artery systolic pressure was found severely increased during the RHC. Treatment included prednisolone, ambrisentan, diuretics, and digoxin. The involvement of the pulmonary artery in patients with idiopathic hypereosinophilic syndrome is an uncommon finding. The patient presents with clinical manifestations of right ventricular systolic dysfunction resulted from severely increased PH.

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