AL (amyloid light-chain) amyloidosis is a plasma cell disorder in which dep
ositions of amyloid light-chain protein cause progressive organ failure. Vi
rtually all patients with AL amyloidosis have a monoclonal protein in the s
erum or urine or a monoclonal population of plasma cells in the bone marrow
. The most common target organ is the kidney and renal amyloidosis manifest
s as proteinuria or nephrotic syndrome in 3/4 of the patients. The median s
urvival is one to two years. It is important to recognize that the amyloido
sis is a dynamic process, and chemotherapy induced reduction of the activit
y of the plasma cell clone reduces the supply of the amyloid precursor prot
ein and can result in a major regression of the deposits. Amyloid-related n
ephrotic syndrome and renal failure are potentially reversible. Conventiona
l-dose melphalan as standard treatment can prolong the median duration of s
urvival about 10 months, but the clinical response rates with improvement o
f impaired organ function are low with a slow response. Upfront high-dose c
hemotherapy with autologous peripheral blood stem cell transplantation is m
uch more effective and can result in a major improvement of the patient's c
linical condition, but the treatment-related toxicity can be relevant due t
o impaired organ function. The initial use of a conventional-dose chemother
apy consisting of vincristine, doxorubicin and dexamethasone (VAD) to achie
ve a remission and subsequent high-dose chemotherapy is the concept of a Ge
rman trial. The improvement of the condition of the patient by this approac
h may increase the tolerability of high-dose chemotherapy and reduce transp
lantation-related problems.