Sachchithanantham et al identified prognostic criteria for patients with monoclonal IgM-related light chain amyloidosis. The authors are from multiple institutions in Europe, Canada and Australia.
Patient selection: monoclonal IgM-related light chain amyloidosis
52% of patients had a lymphoproliferative disorder (malignant lymphoma, CLL).
Prognostic factors:
(1) elevated serum NT-proBNP (> 332 ng/L)
(2) elevated serum troponin T (> 0.035 µg/L)
(3) liver involvement
(4) peripheral neuropathy
Number of Prognostic Factors
Stage
Median Overall Survival
0
I
90 months
1
II
33 months
>= 2
III
16 months
Additional factors associated with survival:
(1) autonomic neuropathy
(2) serum albumin < 30 g/L
(3) higher difference between light chains (> 180 mg/L)
(4) cardiac involvement
(5) Mayo stage (I > II > III)
(6) good response to therapy
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