[27] 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
4	Surgical decortication	37M	Restrictive	Yes	None	Thrombocytopenia

Table 2. Pathologic Features

Patient	Mural thrombus	Endocardial fibrosis	Active myocarditis	Myocardial ischemia	Eosinophil degranulation	CAD
1	Yes	Yes	Marked	Yes	Yes	Absent
2	No	Yes	Marked	No	Yes*	Mild-moderate
3	Yes	Yes	Moderate	Yes	Yes	Mild
4	Yes	Yes	Absent	No	No	Absent

* included numerous Charcot-Leyden crystals; CAD=coronary artery disease


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.
Category: Autopsy

Monday, March 9, 2009 9:30 AM

Poster Session I Stowell-Orbison/Autopsy Award # 10, Monday Morning