Primary (AL, amyloid light-chain) amyloidosis is a plasma cell disorder in
which deposits of amyloid light-chain protein cause progressive organ failu
re. It is important to recognise that amyloidosis is a dynamic process and
chemotherapy-induced reduction of the activity of the plasma cell clone red
uces the supply of the amyloid precursor protein and can result in a major
regression of the deposits. The most common target organ is the kidney and
renal amyloidosis manifests as proteinuria or nephrotic syndrome. Proteinur
ia is seen in three quarters of patients. Amyloid related nephrotic syndrom
e and renal failure are potentially reversible. Fatigue, congestive heart f
ailure, hepatomegaly, peripheral neuropathy, orthostatic hypotension, carpa
l tunnel syndrome and macroglossia are other common features. The median su
rvival is one to two years. Conventional-dose melphalan as standard treatme
nt can prolong the median duration of survival by about ten months, but the
clinical response rates with improvement of impaired organ function are lo
w. Up-front high-dose chemotherapy with autologous peripheral blood stem ce
ll transplantation is much more effective and can result in a major improve
ment in the clinical condition of patients. However, the toxicity related t
o this treatment can be relevant due to impaired organ function. Convention
al-dose chemotherapy consisting of vincristine, doxorubicin and dexamethaso
ne or high-dose dexamethasone or interferon-alpha are other possible approa
ches to treatment. The improvement of patient condition with an effective c
onventional-dose chemotherapy may increase the tolerability of high-dose ch
emotherapy and reduce transplantation related problems.