Interferon α-2a zur Therapie einer diffusen thorakalen Angiomatose im Erwachsenenalter - Ein Fallbericht

Zusammenfassung:

Eine diffuse Angiomatose mit Befall thorakaler Organe ist eine seltene, häufig tödlich verlaufende Erkrankung, welche vor allem im Kindesalter auftritt. Nicht selten wird die definitive Diagnose erst durch Autopsie gestellt. Eine spezifische Therapie ist nicht bekannt. Im vorliegenden Fallbericht schildern wir den Verlauf eines 37-jährigen Patienten mit ausgedehnter diffuser thorakaler Angiomatose unter Interferon-α-2a-Therapie. Während der 18-monatigen Monotherapie mit Interferon α-2a zeigte sich sowohl eine erhebliche Besserung der klinischen als auch der funktionsanalytischen und radiologischen Befunde. Der vorgestellte Fall bestätigt die Bedeutung von Interferon α-2a zur palliativen Therapie aggressiver angiomatöser Krankheitsbilder.

A diffuse angiomatosis affecting thoracal organs is a rare, frequently fatal disorder occurring mainly in childhood. The definite diagnosis is usually made by autopsy. A specific treatment does not exist. In the case report presented here we describe the course of an 37-year old patient presenting initially with dyspnea and hemoptysis. Radiological investigations showed a mass lesion in the anterior superior mediastinum, bihilar masses and a periesophageal mass with soft tissue density and a pericardial effusion. Bilateral interstitial infiltrations were also present. CT-guided mediastinal biopsies and bronchial mucosal biopsies were nonspecific. Open-lung biopsy revealed the diagnosis of an extensive diffuse angiomatosis affecting mediastinum, pericardium and pleura. During the 18-month monotherapy with interferon-α 2a the patient showed significant improvements of clinical as well as laboratory and radiological parameters. The presented case confirms the experience of several authors, that the diagnosis of a diffuse thoracal authors can only be made by open lung biopsy or autopsy. The presented case also underlines the possibility of a palliative therapy of aggressive angiomatous diseases with interferon-α 2a.

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