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Hypereosinophilic syndrome

The hypereosinophilic syndrome is a disease process characterized by a persistently elevated eosinophil count (≥ 1500 eosinophils/mm3) in the blood for at least six months without any recognizable cause after a careful workup, with evidence of involvement of either the heart, nervous system, or bone marrow. more...

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There are two forms of the hypereosinophilic syndrome: Endomyocardial fibrosis and Loeffler's endocarditis. Endomyocardial fibrosis (also known as Davies disease) is seen in Africa and South America, while Loeffler's endocarditis does not have any geographic predisposition.

In both forms of the hypereosinophilic syndrome, the eosinophilia causes infiltration of the myocardium of the heart, which leads to fibrotic thickening of portions of the heart. The portions of the heart most effected by this disease are the apex of the left and right ventricles, fibrotic infiltrations may involve the mitral or tricuspid valves. Because of the infiltrative nature of the disease process, the cavity of the ventricles of the heart diminish in size, causing an obliterative cardiomyopathy and restriction to the inflow of blood in to the chambers of the heart. Ventricular mural thromb may develop.

Chronic eosinophilic leukemia (CEL) is a myeloproliferative disease which shares many common characteristics with hypereosinophilic syndrome. Many cases of CEL have a characteristic gene rearrangement , FIP1L1/PDGFRA, caused by a sub-micoscopic deletion of ~800 thousand base pairs of DNA on chromosome 4. The FIP1L1/PDGFRA fusion gene causes consitutive activation of the platelet derived growth factor receptor - alpha (PDGFRA). FIP1L1/PDGFRA-positive patients respond well to treatment with the tyrosine kinase inhibitor drug, imatinib mesylate (Gleevec® or Glivec®).

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Myiasis due to Hypoderma lineatum infection mimicking the hypereosinophilic syndrome : An article from: Mayo Clinic Proceedings $20.00 Molecular Basis of Chronic Myeloproliferative Disorders $118.42
Hypereosinophilic syndrome: two case reports of contrasting variants.(Review Article) : An article from: Southern Medical Journal $5.95 Hypereosinophilic syndrome or chronic eosinophilic leukemia? / In reply : An article from: Mayo Clinic Proceedings $20.00

Hypereosinophilic syndrome mimicking sepsis and lung injury: dramatic improvement after activated protein C therapy
INTRODUCTION: Hypereosinophilic syndrome (HES) is a systemic illness that usually presents with nonspecific symptoms. However, HES can be fatal, particularly ...
Therapy of hypereosinophilic syndrome, polycythemia vera, atypical CML or CMML with PDGF-R fusion genes, or mastocytosis with Gleevec
This study is currently recruiting patients. Sponsored by M.D. Anderson Cancer Center. The goal of this clinical research study is to see if Gleevec[R], ...
Intravenous mepolizumab in subjects with hypereosinophilic syndromes
This study is currently recruiting patients. Sponsored by GlaxoSmithKline. Hypereosinophilic syndrome (HES) is a rare disease with broad clinical ...
Pulmonary involvement in the hypereosinophilic syndrome
Hypereosinophilic syndrome (HES) is an infiltrative disease of eosinophils affecting multiple organs, including the lung. Hypereosinophilic syndrome ...
Bronchoalveolar Cell Carcinoma Presenting With Acute Respiratory Distress Syndrome And Eosinophilia
Daniel Norman, MSIV; Roham T Zamanian, MD; Thomas T Vovan, MD--UCI Medical Center, Department of Pulmonary and Critical Care, Irvine, California, USA ...
A case of sudden cardiac death due to isolated eosinophilic coronary arteritis
Spontaneous coronary artery dissection is a very rare event and occurs most often in young women following childbirth. It is also known as a rare focal ...
Churg-Strauss syndrome associated with zafirlukast
The leukotriene receptor antagonist zafirlukast (Accolate; Zeneca Pharmaceuticals; Wilmington, Del) recently was approved for use as maintenance therapy ...
A Review of Articles From Last Month's Archives of Pathology & Laboratory Medicine
Listed below are questions based on articles that appeared in last month's print edition of the ARCHIVES. Registered continuing medical education participants ...

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