[Systemic ALlambda amyloidosis associated with vascular fragility]

J Dtsch Dermatol Ges. 2004 Nov;2(11):934-9.
[Article in German]

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.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Amyloidosis / complications
  • Amyloidosis / diagnosis*
  • Amyloidosis / drug therapy*
  • Drug Combinations
  • Eye Hemorrhage / diagnosis*
  • Eye Hemorrhage / etiology
  • Eye Hemorrhage / prevention & control*
  • Facial Dermatoses / diagnosis
  • Facial Dermatoses / etiology
  • Facial Dermatoses / prevention & control
  • Humans
  • Male
  • Melphalan / therapeutic use*
  • Middle Aged
  • Prednisolone / therapeutic use*
  • Treatment Outcome
  • Vascular Diseases / diagnosis*
  • Vascular Diseases / etiology
  • Vascular Diseases / prevention & control*

Substances

  • Drug Combinations
  • Prednisolone
  • Melphalan