A rare cause of severe hepatomegaly with an improving outcome.
A previously healthy 43-year-old man presented with dyspnoea, 15 kg weight loss, severe hepatomegaly and alkaline phosphatase at 5400 U/L. Examinations seemed to suggest cirrhosis, but blood samples did not show any signs of underlying liver disease. Liver biopsy revealed amyloid light chain (AL) amyloidosis and bone marrow showed multiple myeloma (MM). The patient was treated with drugs of choice cyclophosphamide, bortezomib and dexamethasone. He responded well to the treatment and so far achieved partial response. Previously MM was associated with poor prognosis but due to improved treatment for AL the patient can achieve a progression-free period with good quality of life.
Odense University Hospital, Odense C, Denmark.
Line
L
Godskesen
Niels
N
Abildgaard
Jens
J
Kjeldsen
Aleksander
A
Krag
D000328:Adult; D000686:Amyloidosis / Q000188:drug therapy* / Q000209:etiology; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001897:Boronic Acids / Q000008:administration & dosage; D000069286:Bortezomib; D003520:Cyclophosphamide / Q000008:administration & dosage; D003907:Dexamethasone / Q000008:administration & dosage; D006529:Hepatomegaly / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D008297:Male; D009101:Multiple Myeloma / Q000150:complications / Q000188:drug therapy*; D011719:Pyrazines / Q000008:administration & dosage
D001897:Boronic Acids; D011719:Pyrazines; D000069286:Bortezomib; D003907:Dexamethasone; D003520:Cyclophosphamide