Zusammenfassung
□ 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.
Summary
□ This report describes the fatal outcome of a case of adult onset Still’s disease in a 46-year old man. The diagnosis was made according to the 1992 criteria, proposed by Yamaguchi. Nine months after the initial disease manifestations a rapid deterioration with progressive hepatosplenomegaly developed. In parall, pancytopenia and marked hyperferritinemia could be detected. Tranjugular liver biopsy revealed the presence of a hemophagocytic syndrome. The course of the disease was refractory to any form of treatment and the patient died from disseminated intravascular coagulation, hepatic and pulmonary failure.
□ Pathogenetic mechanisms and possible associations between Still’s disease and reactive hemophagocytic syndrome are discussed.
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Zollner, R.C., Kern, P., Steininger, H. et al. Hyperferritinämie beim Still-Syndrom des erwachsenen und reaktives hämophgozytisches Syndrom. Med. Klin. 92, 494–498 (1997). https://doi.org/10.1007/BF03044919
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DOI: https://doi.org/10.1007/BF03044919