[Systemic ALlambda amyloidosis associated with vascular fragility].
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.
Laimer
Martin
M
Universitätsklinik für Dermatologie, Paracelsus Medizinische Privatuniversität Salzburg, Osterreich.
Kaindl
Karin
K
Emberger
Michael
M
Hawranek
Thomas
T
Lanschützer
Christoph M
CM
Bauer
Johann W
JW
Linke
Reinhold P
RP
Mlineritsch
Brigitte
B
Hintner
Helmut
H
D000686:Amyloidosis / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy*; D004338:Drug Combinations; D005130:Eye Hemorrhage / Q000175:diagnosis* / Q000209:etiology / Q000517:prevention & control*; D005148:Facial Dermatoses / Q000175:diagnosis / Q000209:etiology / Q000517:prevention & control; D006801:Humans; D008297:Male; D008558:Melphalan / Q000627:therapeutic use*; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use*; D016896:Treatment Outcome; D014652:Vascular Diseases / Q000175:diagnosis* / Q000209:etiology / Q000517:prevention & control*
D004338:Drug Combinations; D011239:Prednisolone; D008558:Melphalan