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Cardiac Manifestations from Non-FIP1L1-PDGFRα-Associated Hypereosinophilic Syndrome in a 13-Year-Old African American Boy.

Salm CM, Clair NE, Lustig JV, Samyn MM - J Allergy (Cairo) (2010)

Bottom Line: A diagnosis of HES was made after all other etiologies of eosinophilia were excluded.Although he was found to be negative for the FIP1L1-PDGFRα mutation, his cardiac complications included pericardial effusion and restrictive cardiomyopathy, without myocardial necrosis.Multi-organ involvement resulted in pericarditis, pleuritis, nephritis, and dermatitis.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine, Aurora Health Care, Milwaukee, WI 53213, USA.

ABSTRACT
Hypereosinophilic syndrome (HES) is a rare disorder typically seen in males, aged 20 to 50, with a predisposition for Caucasians. It is marked by overproduction of eosinophils (>1,500/μL) and multiorgan system damage due to eosinophilic infiltration and mediator release. There are multiple variants of HES. Cardiac complications are more common in myeloproliferative HES associated with the FIP1L1-PDGFRα mutation. Sequelae range from acute necrosis and thrombus formation to fibrosis of the endomyocardium. We describe a young boy who presented with chest pain and dyspnea. A diagnosis of HES was made after all other etiologies of eosinophilia were excluded. Although he was found to be negative for the FIP1L1-PDGFRα mutation, his cardiac complications included pericardial effusion and restrictive cardiomyopathy, without myocardial necrosis. Multi-organ involvement resulted in pericarditis, pleuritis, nephritis, and dermatitis. In this paper, we review his case and discuss the known subtypes of HES, the classic cardiac complications, and available treatment strategies.

No MeSH data available.


Related in: MedlinePlus

(a) Steady-state free precession cardiac MRI 4 chamber image showing small pericardial effusion, trace pleural effusion, and posterior lung field atelectasis. (b) Delayed enhancement cardiac MRI image (10 minutes postgadolinium infusion) showing no myocardial delayed enhancement; thus, no fibrosis. Visceral and parietal pericardium noted in white with pericardial effusion appearing dark between these two layers.
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fig2: (a) Steady-state free precession cardiac MRI 4 chamber image showing small pericardial effusion, trace pleural effusion, and posterior lung field atelectasis. (b) Delayed enhancement cardiac MRI image (10 minutes postgadolinium infusion) showing no myocardial delayed enhancement; thus, no fibrosis. Visceral and parietal pericardium noted in white with pericardial effusion appearing dark between these two layers.

Mentions: During the initial 4 weeks of hospitalization, serial echocardiograms showed enlargement of the pericardial effusion from small to moderate with myocardial disease with impaired diastolic function (see Figure 1). Serial cardiac enzymes remained normal. Endomyocardial fibrosis was not present on cardiac MRI (see Figure 2). On discharge, the patient had neither chest pain nor shortness of breath. The absolute eosinophil count remained normal.


Cardiac Manifestations from Non-FIP1L1-PDGFRα-Associated Hypereosinophilic Syndrome in a 13-Year-Old African American Boy.

Salm CM, Clair NE, Lustig JV, Samyn MM - J Allergy (Cairo) (2010)

(a) Steady-state free precession cardiac MRI 4 chamber image showing small pericardial effusion, trace pleural effusion, and posterior lung field atelectasis. (b) Delayed enhancement cardiac MRI image (10 minutes postgadolinium infusion) showing no myocardial delayed enhancement; thus, no fibrosis. Visceral and parietal pericardium noted in white with pericardial effusion appearing dark between these two layers.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC2957620&req=5

fig2: (a) Steady-state free precession cardiac MRI 4 chamber image showing small pericardial effusion, trace pleural effusion, and posterior lung field atelectasis. (b) Delayed enhancement cardiac MRI image (10 minutes postgadolinium infusion) showing no myocardial delayed enhancement; thus, no fibrosis. Visceral and parietal pericardium noted in white with pericardial effusion appearing dark between these two layers.
Mentions: During the initial 4 weeks of hospitalization, serial echocardiograms showed enlargement of the pericardial effusion from small to moderate with myocardial disease with impaired diastolic function (see Figure 1). Serial cardiac enzymes remained normal. Endomyocardial fibrosis was not present on cardiac MRI (see Figure 2). On discharge, the patient had neither chest pain nor shortness of breath. The absolute eosinophil count remained normal.

Bottom Line: A diagnosis of HES was made after all other etiologies of eosinophilia were excluded.Although he was found to be negative for the FIP1L1-PDGFRα mutation, his cardiac complications included pericardial effusion and restrictive cardiomyopathy, without myocardial necrosis.Multi-organ involvement resulted in pericarditis, pleuritis, nephritis, and dermatitis.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine, Aurora Health Care, Milwaukee, WI 53213, USA.

ABSTRACT
Hypereosinophilic syndrome (HES) is a rare disorder typically seen in males, aged 20 to 50, with a predisposition for Caucasians. It is marked by overproduction of eosinophils (>1,500/μL) and multiorgan system damage due to eosinophilic infiltration and mediator release. There are multiple variants of HES. Cardiac complications are more common in myeloproliferative HES associated with the FIP1L1-PDGFRα mutation. Sequelae range from acute necrosis and thrombus formation to fibrosis of the endomyocardium. We describe a young boy who presented with chest pain and dyspnea. A diagnosis of HES was made after all other etiologies of eosinophilia were excluded. Although he was found to be negative for the FIP1L1-PDGFRα mutation, his cardiac complications included pericardial effusion and restrictive cardiomyopathy, without myocardial necrosis. Multi-organ involvement resulted in pericarditis, pleuritis, nephritis, and dermatitis. In this paper, we review his case and discuss the known subtypes of HES, the classic cardiac complications, and available treatment strategies.

No MeSH data available.


Related in: MedlinePlus