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. 1997 Aug 1;92(8):494–498. [Article in German] doi: 10.1007/BF03044919

Hyperferritinämie beim Still-Syndrom des erwachsenen und reaktives hämophgozytisches Syndrom

Hyperferritinemia in Still’s disease of the adult and reactive hemophagocytic syndrome

R Christian Zollner 1, Peter Kern 2, Helmuth Steininger 3, Joachim R Kalden 2, Bernhard Manger 2
PMCID: PMC7096029  PMID: 9340475

Abstract

□ Es wird der letale Verlauf eines Still-Syndroms des Erwachsenen bei einem 46 jährigen Patienten beschrieben Die Diagnose wurde nach den 1992 von Yamaguchi vorgeschlagenen Diagnosekriterien gestellt. Etwa neun Monate nach der Erstmanifestation von Fieberschüben kam es zu einer raschen Verschlechterung des Allgemeinbefindens und zunehmender Hepatosplenomegalie. Parallel hierzu trat eine progrediente Panzytopenie und eine ausgeprägte Hyperferritinämie auf. Eine transjuguläre Leberbiopsie erbrachte den histologischen Nachweis eine hämophagozytischen Syndroms. Das Krankheitsbild war therapierefraktär, und es kam zum Auftreten einer Verbrauchskoagulopathie und zur hepatischen und respiratorischen Insuffizienz, an der der Patient verstarb.

□ Der Zusammenhang zwischen Morbus Still und reaktivem hämophagozytischem Syndrom und mögliche pathogenetische Mechanismen werden diskutiert.

Schlüsselwörter: Still-Syndrom, Hyperferritinämie, Reaktives hämophagozytisches Syndrom

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