Table 1.

Extramedullary and extra cardiac features of systemic AL amyloidosis (3). Specific criteria for diagnosis of proteinuria and hepatomegaly are used mostly for research purposes and are subject to refinement and updates.

Organ system	Clinical Manifestations	Diagnostic Criteria
Kidney	Albuminuria; may progress to nephrotic syndrome	1. Proteinuria > 0.5g/24 hours 2. Biopsy
Liver	Hepatomegaly/Splenomegaly	1. Liver edge > 4 cm below the costal margin 2. Serum alkaline phosphatase > 1.5 times upper limit of normal 3. Biopsy
Gastrointestinal tract	Diarrhea Constipation Early satiety Weight loss	1. Biopsy
Nervous system	Ascending, symmetric sensorimotor polyneuropathy Autonomic dysfunction: orthostatic hypotension and gastroparesis	1. Neurological exam 2. Positional BP monitoring 3. Sural nerve biopsy
Pulmonary	Diffuse alveolar infiltrates due to alveolar-septal involvement in systemic AL amyloidosis Nodules and tracheobronchial involvement in localized AL amyloidosis Pleural effusions	1. Biopsy 2. Suggestive chest CT findings in the appropriate clinical setting
Soft tissue	Macroglossia Subcutaneous nodules Rash Bilateral Carpal Tunnel Syndrome Muscle pseudohypertrophy Vascular amyloid: jaw claudication Amyloid lymphadenopathy	1. Physical exam in the appropriate clinical setting 2. Biopsy (rarely required)
Heme: coagulation	Periorbital ecchymosis Bleeding diathesis	1. Abnormal coagulation parameters 2. Factor X levels (most often involved)