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DOI: 10.1055/s-2000-8994
Interferon α-2a zur Therapie einer diffusen thorakalen Angiomatose im Erwachsenenalter - Ein Fallbericht
Interferon-α 2a in the Treatment of Diffuse Thoracic Angiomatosis in Adults. A Case Report:Publikationsverlauf
Publikationsdatum:
31. Dezember 2000 (online)
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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.
Literatur
- 1 Rowen M, Thompson J R, Wiliamson R A, Wood B J. Diffuse pulmonary hemangiomatosis. Radiology. 1978; 127 445-451
- 2 Canny G J, Cutz E, MacLusky I B, Levinson H. Diffuse pulmonary angiomatosis. Thorax. 1991; 46 851-853
- 3 Itri L, Campion M, Dennin R A, Palleroni A V, Guttermann J U, Groopmann J E, Trown P W. Incidence and clinical significance of neutralizing antibodies in patients recieving recombinant interferon alpha-2a by intramuscular injection. Cancer. 1987; 59 668-674
- 4 Koblenzer P J, Bukowski M J. Angiomatosis (hamartomatous hemlymphangiomatosis). Report of a case with diffuse involvement. Pediatrics. 1961; 28 65-76
- 5 Magee F, Wright J L, Peretz D, Donevan R, Churg A. Pulmonary capillary hemangiomatosis. Am Rev Respir Dis. 1985; 132 922-925
- 6 Vevaina J R, Mark J E. Thoracic hemangiomatosis masquerading as interstitial lung disease. Chest. 1988; 93 657-659
- 7 Sidky Y A, Borden E C. Inhibition of angiogenesis by interferons: effects on tumor- and lymphocyte-induced vascular responses. Cancer Res. 1987; 47 5155-5161
- 8 Spiller J C, Sharma V, Woods G M, Hall J C, Seidel F G. Diffuse neonatal hemangiomatosis treated successfully with interferon alfa-2a. J Am Acad Dermatol. 1992; 27 102-104
- 9 Daroca Jr P J, Mansfield R E, Ichinose H. Pulmonary venoocclusive disease: report of a case with pseudoangiomatous features. Am J Surg Pathol. 1977; 1 349-355
- 10 White C W, Sondheimer H M, Crouch E C, Wilson H, Fan L. Treatment of pulmonary hemangiomatosis with recombinant interferon alfa-2a. New Engl J Med. 1989; 320 1197-1211
- 11 Domingo C, Encabo B, Roig J, Lopez D, Morera J. Pulmonary capillary hemangiomatosis: report of a case and review of the literature. Respiration. 1992; 59 178-180
- 12 Masur Y, Remberger K, Hoefer M. Pulmonary capillary hemangiomatosis as a rare cause of pulmonary hypertension. Pathol Res Pract. 1996; 192 (3) 290-295
- 13 Mulliken J B, Zetter B R, Folkman J. In vitro characteristics of endothelium from hemangiomas and vascular malformations. Surgery. 1982; 92 346-353
- 14 White C W, Wolf S J, Korones D N, Sondheimer H M, Tosi M F, Yu A. Treatment of childhood angiomatous diseases with recombinant interferon alfa-2a. J Pediatr. 1991; 118 59-66
- 15 Eltorky M A, Headly A S, Winer-Muram H, Garret H E, Griffin J P. Pulmonary capillary hemangiomatosis: a clinicopathologic review. Ann Thorac Surg. 1994; 57 772-776
- 16 Wu J M, Lin C S, Wang J N, Luo C Y, Hu C Y, Yang H B. Pulmonary cavernous hemangiomatosis treated with interferon alfa-2a. Pediatr Cardiol. 1996; 17 332-334
- 17 Feldman D, Goldstein A L, Cox D C, Grimley P M. Cultured human endothelial cells treated with recombinant leukocyte a interferon: tubuloreticular inclusion formation, antiproliferative effect, and 2', 5'oligoadenylate synthetase induction. Lab Invest. 1988; 58 584-589
- 18 O'Neil E M. Linear sebaceous naevus syndrome with oncogenic rickets and diffuse pulmonary angiomatosis. J Roy Soc Med. 1993; 86 177-178
- 19 Quesada J, Rois R A, Swanson D, Trown P, Guttermann J U. Antitumor activity of recombinant-derived interferon alpha in metastatic renal cell carcinoma. J Clin Oncol. 1985; 3, 11 1522-1528
- 20 Tsuroka N, Sugiyama M, Tawaragi Y, Tsujimoto M, Nishihara T, Goto T, Sato N. Inhibition of in vitro angiogenesis by lymphotoxin and interferon-gamma. Biochem Biophys Res Commun. 1988; 155 429-435
- 21 Friesel R, Komoriya A, Maciag T. Inhibition of endothelial cell proliferation by gamma-interferon. J Cell Biol. 1987; 104 689-696
- 22 Brouty-Boye D, Zetter B R. Inhibition of cell motility by interferon. Science. 1980; 208 516-518
- 23 Heyns A D, Eldor A, Vlodavsky I, Kaiser N, Fridman R, Panet A. The antiproliferative effect of interferon and the mitogenic activity of growth factors are independent cell cycle events: studies with vascular smooth muscle cells and endothelial cells. Exp Cell Res. 1985; 161 297-306
- 24 Holden K R, Alexander F. Diffuse neonatal hemangiomatosis. Pediatrics. 1970; 46, 3 411-421
Dr. med. Andreas Eichler
Medizinische Universitätsklinik Innere Medizin V
66421 Homburg