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[Systemic ALlambda amyloidosis associated with vascular fragility].

Authors
  • Laimer, Martin
  • Kaindl, Karin
  • Emberger, Michael
  • Hawranek, Thomas
  • Lanschützer, Christoph M
  • Bauer, Johann W
  • Linke, Reinhold P
  • Mlineritsch, Brigitte
  • Hintner, Helmut
Type
Published Article
Journal
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
Publication Date
Nov 01, 2004
Volume
2
Issue
11
Pages
934–939
Identifiers
PMID: 16281613
Source
Medline
License
Unknown

Abstract

A 63-year-old patient had experienced bilateral spontaneous periorbital hemorrhage for one year. After hospitalization because of recurring hemoptysis, biopsies of skin and colon revealed systemic ALlambda amyloidosis. In addition, the heart and lungs appeared to be involved. Monoclonal gammopathy was excluded by a normal plasma cell count and a polyclonal expression pattern in a bone marrow sample and by radiographic examination. The patient was treated with a relatively non-aggressive regimen of melphalan and prednisolone monthly with careful hematologic monitoring. This approach led to a significant improvement in relevant parameters. Recent advances in diagnosis, monitoring and therapy have made it easier to manage patients with amyloidosis with their poor prognosis.

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