AL-amyloidosis is one of the most common amyloidoses and can be found in a
localized and a systemic form. The precursor protein is an immunoglobulin l
ight chain which as AL-protein in both localized and systemic AL-amyloidosi
s shows the same pattern of fragmentation and changes of primary structure.
In this work it is shown that that there is a difference between localized
and systemic amyloidosis in respect to accompanying giant cells which cons
tantly are found associated with amyloid deposits in localized AL-amyloidos
is. In addition, giant cells were found together with amyloid deposits in l
ymph nodes of some cases of systemic AL-amyloidosis. Based on these finding
s and electron microscopic studies, it is discussed whether the giant cells
actively participate in amyloid fibril formation by uptake and modificatio
n of the precursor protein or the giant cells are part of a foreign body re
action. Included in this work are two new cases of localized lung (lambda I
) and ureteric (rd) AL-amyloidosis.