LOEFFLER’S EOSINOPHILICENDOCARDITIS

DEFINITION

RestrictiveCardiomyopathy

Eosinophilia( > 120×10ᶺ9/L)

Disease of the heart muscle Impaired ventricular filling Diastolic volume is NORMAL or

INCREASED Affects ONE or BOTH ventricles

stage of manifestation

Pathologic specimens Clinical manifestations

Necrotic stage (early stage)

Eosinophilic myocarditis

Endomyocardial fibrosis

Hypereosinophilia:• Fever, Sweating, Weight loss, Cough, Rash,

Chest pain, Lymphadenopathy, Splenomegaly

Acute carditis: Anorexia, Weight loss, cough, Pulmonary

infiltrates, Skin and retinal lesion, AV valve regurgitation, Biventricular failure, Polymorphic ventricular tachycardia

 Thrombotic stage Clinical manifestations of

thromboembolismThrombotic emboli: Cerebral, splenic, renal, and coronary

infarction, Splinter hemorrhages 

Fibrotic stage (late stage)

Acute Heart Failure Restrictive myopathy : AV valvular regurgitation,Kussmall sign, elevated jugular venous pressure, pulmonary edema, murmur of mitral regurgitation

 

ETIOLOGY

hypereosinophilia

ACCOMPANIED BY:

Hyper eosinophilic syndromeEosinophilic leukemia

CarcinomaLymphoma

Drug reactions Parasites

GROSS APPEARANCE OF ENDOCARDIUM

• Normal heart • Dense fibrosis of ventricles and valve movement

PATHOGENESIS

Eosinophilic penetration• Degranulation

of basic proteins

• Cytotoxic effects inhibiting multiple enzyme systems

Fibrotic thickening of portions of heart• Cationic

proteins + anionic endothelium

• Endothelial and myocyte damage

Large mural thrombi• Impaired

anticoagulation activity

• ↑ endocardial thrombus formation

LABORATORY INVESTIGATIONS

1.Complete blood count - eosinophilia

2.Cytogenetics – FISH3.Molecular analysis – FIP1L1-

PDGFRA fusion gene4.Percutaneous Endomyocardial

Biopsy - collagen

IMAGING

1.ECG2.ECHOCARDIOGRAPHY3.CT and MRI

PHARMACOLOGICAL MANAGEMENT Group of drugs Comments

Diuretics, Cardiac Glycosides, ACE Inhibitors

Heart failure drugs for symptomatic relief

Tyrosine Kinase Inhibitors Imanitib – inhibits cell proliferation

Anti IL5 monoclonal AB Mepolizumab – inhibit production/activation/maturation of eosinophils

Antineoplastic and Antimetabolites Hydroxyurea – inhibit cell growth and proliferation

Corticosteroids Methylprednisolone – anti-inflammatory drugs

Interferon therapy Interferon alpha 2B

SURGICAL MANAGEMENT

Endocardioectomy

FOLLOW UP

Complete blood count Echocardiography

DIFFERENTIAL DIAGNOSIS

1. Cardiac Neoplasms2. Cardiomyopathies

PROGNOSISPoor

CAROL TAFADZWA DZORANI 631D