Zusammenfassung
□ Hintergrund
Die Symptome des Morbus Moschcowitz waren bei Erstbeschreibung charakterisiert durch eine thrombozytopenische Purpura und hämolytische Anämie. Daneben umfaßt der Morbus Moschcowitz ein klinisch buntes Bild von Organmanifestationen (neurologische, renale, aber auch gastrointestinale oder kardiale Beteiligung). Auch aufgrund der Seltenheit der Erkrankung sind die Überlegungen zur Pathogenese uneinheitlich. Die abgeleiteten Therapieversuche sind kaum durch kontrollierte Studien zu untersuchen, und ihre Bewertung bleibt schwierig.
□ Schlußfolgerung
Neben vielen weiteren Therapieansätzen scheint derzeit die Plasmatherapie (Gabe von Fresh-frozen-Plasma oder Austauschtherapie) die wichtigste konservative Behandlungsmöglichkeit. In therapierefraktären Situationen kann die chirurgische Option einer Splenektomie hilfreich sein.
Abstract
□ Background
The symptoms primarily described in Morbus Moschcowitz included thrombocytopenic purpura and hemolytic anemia. In addition it presents with a variety of clinical manifestations depending on the organs involved (i. e. neurological, renal, gastrointestinal, cardiac involvement). It is a rare disease and the pathogenesis still remains unclear. The efficacy of derived therapeutical concepts can hardly be assessed in controlled trials.
□ Conclusion
Currently the main option seems to be plasma therapy. In nonresponders surgical procedures (splenectomy) may be of benefit to the patient.
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Jakob, A.R., Hiller, E. Multiorganbeteiligung bei rezidivierendem Morbus Moschcowitz. Med Klin 93, 624–626 (1998). https://doi.org/10.1007/BF03042678
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DOI: https://doi.org/10.1007/BF03042678