Chinese consensus on the diagnosis and treatment of immunoglobulin light-chain cardiac amyloidosis

[...]amyloid fibers show a disorderly arrangement of unbranched fiber structures with a diameter of 7–14 nm under an electron microscope, which can be used as a complementary diagnostic means. Diagnostic criteria of AL-CA The diagnosis of AL-CA needs to meet the following (1) + (2) + (3) conditions and at least one of (4) or (5): (1) Typical clinical manifestations or imaging characteristics of AL-CA; (2) Monoclonal immunoglobulin in the serum or/and urine;[2] (3) The tissue biopsy of myocardium or other parts exhibits deposition of amorphous material that is Congo red positive under polarizing light microscopy or non-branching fiber with a diameter of 7–14 nm under an electron microscope; (4) The amyloidogenic protein component is proven to be immunoglobulin light chain based on immunohistochemistry, immunofluorescence, immune-electron microscopy or mass spectrometry;[1] (5) A 99mTc-labeled pyrophosphate (99mTc-PYP) scan of the heart is negative. [...]available treatments target clonal plasma cells to reduce serum monoclonal immunoglobulin levels and achieve cardiac remission through the body’s self-clearance mechanism. [...]the goal of AL-CA treatment is high-quality hematological remission (at least very good partial response [VGPR] and above). First-line treatment Daratumumab: (1) Because daratumumab can induce a rapid and deep hematological response, it is recommended as the first-line therapy.