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🌱 來自: amyloidosis

diagnostic studies of amyloidosis

• Biopsy (abd fat pad, rectal, etc.):

• apple- green birefring w/ Congo red (Se <85% Sp >90%)

• If suspect AL:

  • FLC
  • SPEP & UPEP + immunofixation, free light chains (FLC), ± BM biopsy

• If suspect renal involvement:

  • U/A for proteinuria
  • abd fat pad bx

• If suspect CMP:

  • ECG (↓ volt, conduction abnl),
  • TTE (↓ longitudinal strain, LVH, valve & septal thickening, myocard. speckling), MRI (late gad. enhancement).
  • R/o plasma cell dyscrasia; if ⊖ → (99m)Tc-pyrophosphate (PYP) SPECT for TTR. ± Cardiac bx.

• Mass spec of biopsy to ID fibril type. Genetic testing to distinguish wt vs. hereditary ATTR.

• International Myeloma Working Group diagnostic criteria for systemic AL amyloidosis

• ddx: multiple myeloma staging systems

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