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Table 1 Updated definition of organ involvement in AL amyloidosis

From: AL Amyloidosis

Involved organ or tissue

Criteria

Kidney

24-hr urine protein > = 0.5 g/day, predominantly albumin

Heart

NT-proBNP > 332 ng/l (in the absence of renal failure or atrial fibrillation) or mean wall thickness in diastole by echography > 12 mm, no other cardiac cause

Liver

Total liver span > 15 cm in the absence of heart failure, or alkaline phosphatase > 1.5 times institutional upper limit of normal

Nerve

Peripheral: Symmetric lower extremity sensorimotor peripheral neuropathy

 

Autonomic: gastric-emptying disorder, pseudo-obstruction,voiding dysfunction not related to direct organ infiltration.

Gastro-intestinal tract

Direct biopsy verification with symptoms

Lung

Direct biopsy verification with symptoms

 

Interstitial radiographic pattern

Soft tissues

Tongue enlargement, arthropathy, claudication (presumed vascular amyloid), skin lesions, myopathy (by biopsy or pseudohypertrophy), lymph node (may be localized), carpal tunnel syndrome

  1. From Gertz et al. ( 3,4) with permission.