Table 1.

Diagnostic assessment of patients with PN and monoclonal gammopathy

1. Complete medical history 
2. Physical/neurological exam 
3. PN characterization: nerve conduction studies and electromyography 
4. Monoclonal gammopathy characterization:
a. Renal function, serum calcium, and complete blood count
b. Serum/urine protein electrophoresis and immunofixation
c. Serum Ig levels
d. Serum free light chain levels
e. 24-h urine excretion
f. Bone marrow aspirate and/or biopsy: morphology, flow cytometry, molecular and cytogenetic analysis based on findings, such as MYD88 L265P mutation analysis in IgM MGUS, and FISH for t(11;14) in AL amyloidosis 
5. Other investigations according to previous findings:
a. Serum cobalamin levels
b. Cryoglobulins
c. Testing for HIV, HCV, and, if indicated, syphilis and Lyme disease
d. Autoantibodies: antinuclear antibodies, anti-MAG, and, if required, anti-HNK1, anti-GM1, disialosil ganglioside or anti-sulfatides antibodies
e. Serum levels of VEGF
f. Endocrine screening of the 4 major endocrine axes: gonadal, thyroid, glucose metabolism, and adrenal
g. Imaging assessment: skeletal survey, MRI, CT, or positron emission tomographic CT
h. Cardiac evaluation: serum troponin and NT-proBNP levels, electrocardiogram, echocardiography, and/or MRI
i. Fat pad aspiration with Congo red staining and, if positive, amyloid typing
j. Nerve biopsy 
1. Complete medical history 
2. Physical/neurological exam 
3. PN characterization: nerve conduction studies and electromyography 
4. Monoclonal gammopathy characterization:
a. Renal function, serum calcium, and complete blood count
b. Serum/urine protein electrophoresis and immunofixation
c. Serum Ig levels
d. Serum free light chain levels
e. 24-h urine excretion
f. Bone marrow aspirate and/or biopsy: morphology, flow cytometry, molecular and cytogenetic analysis based on findings, such as MYD88 L265P mutation analysis in IgM MGUS, and FISH for t(11;14) in AL amyloidosis 
5. Other investigations according to previous findings:
a. Serum cobalamin levels
b. Cryoglobulins
c. Testing for HIV, HCV, and, if indicated, syphilis and Lyme disease
d. Autoantibodies: antinuclear antibodies, anti-MAG, and, if required, anti-HNK1, anti-GM1, disialosil ganglioside or anti-sulfatides antibodies
e. Serum levels of VEGF
f. Endocrine screening of the 4 major endocrine axes: gonadal, thyroid, glucose metabolism, and adrenal
g. Imaging assessment: skeletal survey, MRI, CT, or positron emission tomographic CT
h. Cardiac evaluation: serum troponin and NT-proBNP levels, electrocardiogram, echocardiography, and/or MRI
i. Fat pad aspiration with Congo red staining and, if positive, amyloid typing
j. Nerve biopsy 

FISH, fluorescence in situ hybridization; HCV, hepatitis C virus; MRI, magnetic resonance imaging; NT-proBNP, N-terminal pro b-type natriuretic peptide.

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