Eosinophilic Endomyocardial Disease: A Clinicopathological Study of Autopsy and Surgical Heart Specimens
AP Sciallis, DJ Firchau, WD Edwards, DV Miller. Mayo Clinic, Rochester, MN
Background: Eosinophilic endomyocardial disease (EoEMD), also known as Leffler's or tropical endomyocardial disease, is a rare cardiac disorder associated with prolonged eosinophilia of any cause. In the temperate zones, the most common association is with hypereosinophilic syndrome, whereas in tropical locations, helminth-induced hypereosinophilia is usually the etiology. Clinically, most patients develop restrictive cardiomyopathy or atrioventricular valve insufficiency. Ventricular mural thrombus may also be present. Endomyocardial biopsy is regarded as the gold standard for diagnosis. The aim of this study was to characterize more fully the clinical and pathologic spectrum of changes found in EoEMD from autopsy and larger surgical specimens, rather than from endomyocardial biopsy tissues.
Design: We searched the pathology files of the Mayo Clinic Tissue Registry for cases of EoEMD (1980-2008). Of the 79 cases, 2 were from autopsies, 1 from surgical explantation, and 1 from surgical decortication/thrombectomy. Clinical, gross, and microscopic findings of each case were systemically reviewed.
Results: Clinical and pathologic data are presented in Tables 1 and 2, respectively.
Table 1. Clinical Data
|Patient||Source||Age, Sex at Examination||Type of Cardio-myopathy||Peripheral Blood Eosinophilia||History of Peripheral Thrombus||Relevant Medical History|
|1||Autopsy||42M||Restrictive||Yes||Portal vein thrombosis, pulmonary emboli||Hypereosinophilic syndrome|
|2||Surgical explant||46F||Dilated||Yes||Pulmonary emboli||Severe asthma|
|3||Autopsy||74F||Restrictive||No||Pulmonary emboli||Rheumatoid arthritis|
Table 2. Pathologic Features* included numerous Charcot-Leyden crystals; CAD=coronary artery disease
|Patient||Mural thrombus||Endocardial fibrosis||Active myocarditis||Myocardial ischemia||Eosinophil degranulation||CAD|
Conclusions: While generally congruent with recognized features of EoEMD, this study highlights the variability both in the clinical manifestations (dilated cardiomyopathy; late-phase disease without peripheral eosinophilia), and in the histopathologic findings (2 cases with patchy ischemic change likely due to global hypoperfusion or microthrombi; another case with massive Charcot-Leyden crystals) seen in this rare disorder.
Monday, March 9, 2009 9:30 AM
Poster Session I Stowell-Orbison/Autopsy Award # 10, Monday Morning