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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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A Review of Articles From Last Month's Archives of Pathology & Laboratory Medicine
From Archives of Pathology & Laboratory Medicine, 3/1/05

Listed below are questions based on articles that appeared in last month's print edition of the ARCHIVES. Registered continuing medical education participants should use the March 2005 answer sheet to answer these questions.

1. Eosinophilic infiltration of the esophageal mucosa is not a specific diagnostic feature of gastroesophageal reflux disease (GERD).

True or False?

(from Is There a Set of Histologie Changes That Are Invariably Reflux Associated?-Takubo et al)

2. The cytokeratin expression of gastric cardia mucosa is:

a. CK7 negative CK20 negative

b. CK7 positive CK20 negative

c. CK7 negative C20 positive

d. CK7 positive CK20 positive

e. CK8 positive CK19 negative

(from Is Adenocarcinoma of the Esophagogastric Junction or Cardia Different From Barrett Adenocarcinoma?-Ectors et al)

3. Monophasic spindle subtype of synovial sarcomas has immunohistochemical and ultrastructural evidence of mesenchymal differentiation.

True or False?

(from Primary Renal Synovial Sarcoma Confirmed by Cytogenetic Analysis-Shannon et al)

4. The most common inherited risk factor for venous thromboembolism is:

a. factor VIII deficiency

b. familial hemochromatosis

c. diabetes mellitus

d. familial hyperlipidemia

e. factor V Leiden with activated protein C resistance

(from Extensive Aortic Thromboembolism Due to Acquired Hypercoagulable State-Zhang et al)

5. Estrogen receptors are detectable in the prostatic urethra.

True or False?

(from Estrogen Receptor Expression in Papillary Urothelial Carcinoma of the Bladder and Ovarian Transitional Cell Carcinoma-Croft et al)

6. Systemic mast cell disease is frequently associated with:

a. myeloproliferative disorders

b. hypereosinophilic syndrome

c. myelodysplastic syndromes

d. a and b

e. a and c

(from Evaluation of Mast Cells in Myeloproliferative Disorders and Myelodysplastic Syndromes-Dunphy)

Copyright College of American Pathologists Mar 2005
Provided by ProQuest Information and Learning Company. All rights Reserved

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