Zeitschrift für Kardiologie

, Volume 94, Issue 11, pp 761–766 | Cite as

Hypereosinophilic syndrome associated with acute necrotizing myocarditis and cardiomyopathy

  • M. Huntgeburth
  • M. Lindner
  • J. W. U. Fries
  • U. C. Hoppe
CASE REPORT

Summary

We report the rare case of a 55-year-old female with massive eosinophilic myocarditis and severe, however reversible, impairment of left ventricular function. The patient presented with reduced physical condition, progressive dyspnea on exertion and peripheral edema. The white blood count revealed a leukocytosis and markedly elevated peripheral blood eosinophilics (48.8%). An endomyocardial biopsy demonstrated massive myocardial infiltration with eosinophilic granulocytes and necrosis. The symptoms and laboratory parameters indicate the presence of a hypereosinophilic syndrome. The differential diagnosis of a Churg-Strauss syndrome is discussed. Medical heart failure treatment according to international guidelines and an immunosuppressive treatment with prednisolone (Decortin H® 1.5 mg/kgBW) were initiated. This therapy led to a dramatic reduction of the eosinophilic granulocyte count and normalization of the peripheral blood count, which correlated with a significant improvement of clinical symptoms. Consistently, an increase of left-ventricular function was observed. Upon successive dose reduction to a maintenance dosage of 10 mg prednisolone, the patient’s clinical status and peripheral blood count remained stable.

Key words

Eosinophilia idiopathic hypereosinophilic syndrome myocarditis cardiomyopathy endomyocardial biopsy steroids 

Hypereosinophiles Syndrom mit akut nekrotisierender Myokarditis und Kardiomyopathie

Zusammenfassung

Wir berichten über eine 55-jährige Patientin mit einer ausgeprägten eosinophilen Myokarditis und hochgradig eingeschränkter linksventrikulärer Pumpfunktion. Klinisch manifestierte sich die Erkrankung mit reduziertem Allgemeinzustand, zunehmender Belastungsdyspnoe und peripheren Ödemen. Im Blutbild fand sich eine Leukozytose mit ausgeprägter peripherer Eosinophilie (48,8%). Eine endomyokardiale Biopsie zeigte eine massive Infiltration des Myokards mit eosinophilen Granulozyten und Nekrosen. Die Symptome und Laborparameter sprechen am ehesten für ein hypereosinophiles Syndrom. Die Differentialdiagnose eines Churg-Strauss-Syndroms wird diskutiert. Es wurde eine leitliniengerechte medikamentöse Herzinsuffizienztherapie sowie immunsuppressive Therapie mit Prednisolon (Decortin H®, 1,5 mg/kgKG) eingeleitet. Darunter lies sich eine rasche Reduktion der eosinophilen Granulozyten mit Normalisierung des peripheren Blutbildes beobachten, was mit einer deutlichen Verbesserung der klinischen Symptomatik korrelierte. Insbesondere war eine signifikante Zunahme der linksventrikulären Pumpfunktion zu beobachten. Nach langsamer Dosisreduktion auf eine Erhaltungsdosis von 10 mg Prednisolon blieben das klinische Beschwerdebild und Blutbild im Verlauf stabil.

Schlüsselwörter

Eosinophilie idiopathisches hypereosinophiles Syndrom Myokarditis Kardiomyopathie Endomyokardiale Biopsie Steroide 

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Copyright information

© Steinkopff-Verlag 2005

Authors and Affiliations

  • M. Huntgeburth
    • 1
  • M. Lindner
    • 1
  • J. W. U. Fries
    • 2
  • U. C. Hoppe
    • 1
  1. 1.Klinik III für Innere MedizinUniversität zu KölnCologneGermany
  2. 2.Universität zu Köln, Institut für PathologieCologneGermany

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