Management der Langerhans-Zell-Histiozytose bei erwachsenen Patienten

Zusammenfassung

Die Langerhans-Zell-Histiozytose ist eine seltene myeloische Neoplasie aus dem Formenkreis der Histiozytosen. Durch entzündliche Gewebezerstörung mit Fibrosierung kann es in jedem Organ zu einer Dysfunktion kommen. Am häufigsten sind Skelett, Lungen und Haut betroffen. Symptome, insbesondere eine Hypophyseninsuffizienz, gehen der Diagnosestellung häufig Monate bis Jahre voraus. Der Krankheitsverlauf ist variabel und reicht von spontanen Rückbildungen bis hin zu chronisch rezidivierenden Verläufen. Die Therapiemaßnahmen richten sich nach Krankheitsaktivität, betroffenen Organen und Organdysfunktion. Aufgrund häufig zugrunde liegender somatischer Genmutationen im MAPK-Signaltransduktionsweg stehen jetzt auch gezielt wirksame Inhibitoren zur Verfügung. Die Prognose der Patienten ist günstig. Erkrankung oder Therapiemaßnahmen können jedoch zu irreversiblen Spätfolgen führen. Eine vor allem symptomorientierte Nachsorge ist empfehlenswert.

Abstract

Langerhans cell histiocytosis is a rare myeloid neoplasia from the group of histiocytoses. Inflammatory tissue destruction with fibrosis can cause dysfunction in any organ. The skeleton, lungs, and skin are most commonly affected. Symptoms, especially of pituitary insufficiency, often precede diagnosis by months to years. The course of the disease is variable, ranging from spontaneous regressions to chronic recurrent courses. Therapeutic measures depend on disease activity, affected organs and organ dysfunction. Due to frequently underlying somatic gene mutations in the MAPK signal transduction pathway, targeted inhibitors are now available. The prognosis of patients is favorable. Nevertheless, disease or therapy can lead to irreversible late effects. A primarily symptom-oriented follow-up is recommended.

Schlüsselwörter

Langerhans-Zell-Histiozytose - erwachsene Patienten - Diagnostik - Therapie - Prognose

Keywords

langerhans cell histiocytosis - adult patients - diagnostic - therapy - prognosis

Publication History

Article published online:
03 February 2023

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