Kyle et al divided patients with primary systemic amyloidosis into 3 risk groups based on the presence or absence of 5 risk factors. The risk group correlates with 1 year survival. The authors are from the Mayo Clinic.
Risk factors:
(A) congestive heart failure
(B) urine monoclonal light chains
(C) hepatomegaly
(D) multiple myeloma
(E) monoclonal serum protein
There are 32 possible combinations of these risk factors.
Combination |
Risk Group |
none |
low |
A |
high |
B |
moderate |
C |
moderate |
D |
moderate |
E |
low |
A + B |
high |
A + C |
high |
A + D |
high |
A + E |
moderate |
B + C |
high |
B + D |
high |
B + E |
low |
C + D |
high |
C + E |
low |
D + E |
low |
A + B + C |
high |
A + B + D |
high |
A + B + E |
high |
A + C + D |
high |
A + C + E |
high |
A + D + E |
high |
B + C + D |
high |
B + C + E |
moderate |
B + D + E |
moderate |
C + D + E |
moderate |
A + B + C + D |
high |
A + B + C + E |
high |
A + B + D + E |
high |
A + C + D + E |
high |
B + C + D+ E |
high |
A + B + C + D + E |
high |
where:
• The risk assignments are based on the text on page 222. However, the text used to describe the combinations is sometimes ambiguous. especially when there are 2 risk factors.
High risk:
(1) any combination with "A" except A + E
(2) two risk factors without A or E (B + C, B + D, C + D)
(3) B + C + D
(4) B + C+ D + E
Low risk:
(1) none
(2) E
(3) two risk factors including E
Moderate risk: all other combination
Risk Group |
Survival at 1 Year |
low |
75% |
moderate |
54% |
high |
15% |
Specialty: Hematology Oncology