Background. Retrospective study about results of treatment of patients (pts) with primary (AL) and secondary (AA)
amyloidosis is given. 31 pts with systemic forms of amyloidosis have been treated and followed-up in our department
Methods and Results. 6 men and 11 women were in the AL group with the mean age of 59 years. Multiple myeloma
was diagnosed in 9 pts, monoclonal gammapathy of undetermined significance (MGUS) was found in 8 pts. The
kidneys were affected in all pts, heart in 59 % of pts, liver, joints and skin in 26 % of pts and polyneuropathy was
detected only in 1 pt. Progression of renal insufficiency with decrease of glomerular filtration rate (GFR) was detected
in the AL group at the end of follow-up period comparedwith the initial level (p<0.05) despite the intensive treatment.
The difference did not reach statistical significance in other investigated parameters. Median of survival was 13
months from the assessment of diagnosis. Partial remission of amyloidosis was achieved in 9 pts, stable disease was
in 5 pts and in 3 pts the disease progressed. 4 men and 10 women were in the AA group with mean age of 58 years.
Underlying disease was rheumatoid arthritis in 7 pts, ankylosing spondylitis in 2 pts, juvenile chronic arthritis in 1
pt, Crohn´s disease in 2 pts, eosinophilic fasciitis in 1 pt and chronic abscesses in NK cell deficiency in 1 pt. The
kidneys were affected in all pts, bowels and heart in 36 % of pts. GFR (p<0.05) and plasma creatinine (p<0.01)
significantly decreased at the end of follow-up period compared with initial levels.Median of survival was 30months.
Partial remission was achieved in 2 pts, stable disease was in 3 pts and progression was detected in 9 pts despite the
use of various treatment regimens.
Conclusions. Both forms of systemic amyloidosis represent severe disease with limited response to treatment. The
use of new drugs is promising and could lead to better response to treatment.
AL amyloidosis, AA amyloidosis, MGUS, paraprotein, ASCT, immunosuppressive drugs.