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Related Nova Publications
127
inflammation; and Aβ, which is found in patients on long-term haemodialysis
for renal failure. Amyloid deposits may affect many organs (systemic
amyloidoses), thus decreasing the patient’s survival if vital organs are affected,
or they may occur in a single tissue (localized amyloidoses) with a better
prognosis. Biopsy is necessary to confirm the amyloid deposit, which acquires
an apple green birefringence under polarized light after staining with Congo
red. Abdominal fat aspirate is a non-invasive technique that presents high
sensibility, 70-90%. Prompt diagnosis and evaluation for systemic disease is
essential prior to treatment.
SERUM AND URINE FREE LIGHT CHAIN ANALYSIS IN
EVALUATION OF AMYLOIDOSIS AL AND OTHER
MONOCLONAL GAMMOPATHIES
*
Stanley S. Levinson
†
Laboratory Service, Department of Veterans Affairs Medical Center,
Louisville, KY
The Department of Pathology and Laboratory Medicine, School
of Medicine, University of Louisville, Louisville, KY
This article discusses approaches for testing monoclonal proteins in
clinical laboratories with special emphasis on amylodosis AL and free light
chain disease. Where reflex testing is possible good communication between
the laboratory and the clinician leads to the most effective work-up. The article
briefly reviews the major monoclonal gammopathies. including multiple
myeloma, lymphocytoplasmic lymphoma, heavy chain disease, plasmacytoma,
plasma cell dyscrasia associated with neuropathy, amyloidosis AL, free light
chain disease, and monoclonal gammopathy of unknown significance. There is
emphasis on the pathophysiology of amyloidosis AL and free light chain
disease and a testing algorithm for identifying amyloidosis AL. Standard
*
The full version of this chapter can be found in Amyloids: Composition, Functions and
Pathology, edited by Irene P. Halcheck and Nancy R. Vernon, published by Nova Science
Publishers, Inc, New York, 2012.
†
Correspondence: Stanley S. Levinson, at Laboratory Service. Voice:(502) 895-3401
X5565/5572, Fax: (502) 894-6265, E mail:levinson@louisville.edu.
Related Nova Publications
128
clinical laboratory techniques for identifying monoclonal proteins are
discussed and examples of serum and urine protein electrophoresis and
immunofixation electrophoresis are shown. A new serum free light chain assay
that adds additional armament to traditional testing is discussed. The article
discusses the strengths and weaknesses of this assay as compared to traditional
urine testing for monoclonal free light chains. It is concluded with an
algorithm that addresses approaches for monoclonal protein screening.
INDEX
#
11-1F4, 38, 39, 40, 49
2A4, 39, 40
A
AL amyloidosis, viii, x, xi, 2, 3, 4, 6, 7, 8, 9,
15, 16, 17, 19, 20, 22, 23, 24, 26, 27, 28,
30, 32, 40, 41, 42, 43, 44, 49, 52, 53, 54,
56, 61, 68, 71, 82, 91, 92, 93, 94, 95, 96,
97, 98, 100, 101, 102, 103, 104, 105, 119
ALN-TTR02, 34, 65
ALN-TTRSc, 34
amyloid, v, vii, viii, ix, x, 1, 2, 3, 4, 5, 6, 8,
9, 10, 12, 14, 16, 18, 19, 20, 21, 22, 23,
25, 26, 27, 28, 29, 30, 31, 32, 33, 35, 36,
37, 38, 39, 41, 42, 43, 44, 45, 46, 47, 48,
49, 51, 52, 54, 55, 56, 57, 58, 61, 62, 65,
66, 67, 68, 69, 70, 71, 73, 74, 75, 76, 77,
78, 79, 80, 81, 82, 83, 84, 85, 86, 87, 88,
89, 90, 91, 93, 97, 98, 102, 103, 107,
122, 126
amyloid deposits, 37, 74, 80, 126
amyloidosis, 1, iii, v, 15, 18, 20, 22, 27, 29,
31, 41, 42, 43, 45, 49, 51, 53, 57, 58, 63,
64, 67, 69, 70, 71, 88, 89, 91, 94, 95,
102, 103, 104, 111, 116, 117, 120, 125,
126, 127
Antisense oligonucleotide drugs, 35
B
Bence-Jones protein, 94, 95, 99
biopsy, 56, 89, 127
C
cardiac enzymes, 96
cardiac involvement, 2
cardiomyopathy, v, vii, 1, 4, 72, 123
Congo red, ix, 6, 31, 43, 56, 57, 66, 73, 76,
77, 78, 79, 80, 82, 83, 85, 86, 87, 88, 89,
93, 127
Congo red fluorescence, 43, 82
CPHPC, 18, 36, 37, 89
D
desorption, 86, 90
diabetes mellitus II, 75
E
echocardiography, viii, 2, 6, 10, 12, 14, 94,
102
electrocardiography, 9, 23
electrophoresis, 99
Index
130
F
familial amyloid cardiomyopathy, 4, 33
formalin-fixed paraffin embedded (FFPE)
material, 80, 83, 84, 85, 86
free light immunoglobulin chains (FLC), x,
6, 16, 91, 93, 94, 95, 97, 98, 99, 100, 102
FreeLite
TM
, x, 92, 93, 99
G
granular sparkling, 10
H
heavy/light chain pairs of immunoglobulins
(HLC), x, xi, 92, 96, 101, 102
HevyLite
TM
, x, 92, 96
histopathology, 75, 118
I
Ig-lambda positivity, 83
immuno-electron microscopy, 84, 90
immunofixation, 99
immunohistochemical (IHC) staining, 38,
80, 81, 82, 83
immunotherapy, v, 29, 36, 48
immunoturbidimetry, x, 92
IONIS-TTR
Rx
, 35
Islet of Langerhans, 75
L
laser microdissection, 22, 57, 58, 66, 71, 85,
86, 88, 89
laser microdissection-liquid
chromatography/mass spectrometry, 85
late gadolinium enhancement, 14
M
magnetic resonance, viii, 2, 14, 15
Mass spectrometry, 85, 90
matrix, 86, 90
matrix XE "matrix" -assisted laser
desorption XE "desorption" /ionization,
86, 90
monoclonal gammopathies, x, xi, 3, 52, 91,
92, 93, 96, 102, 103, 104, 115, 127
monoclonal immunoglobulin, x, 6, 91, 92,
93, 94, 99, 104
monoclonal immunoglobulin light chains, x,
91, 93
multidimensional protein identification
technology, 85
N
natriuretic peptides, x, 91, 92, 94, 95
NEOD001, 40, 49
NT-proBNP, 7, 8, 9, 16, 17, 23, 40, 96, 97,
101, 104
P
pancreatic tissue, 75
patisiran, 34, 65
polarizing microscopy, 56, 77, 78, 79
protein misfolding, 52, 75, 88
pseudo-infarct pattern, 9
R
revusiran, 34
S
scintigraphy, 13, 24, 25, 36, 37
Senile systemic amyloidosis, 4, 21
serum amyloid P component, ix, 12, 24, 25,
27, 31, 36, 46, 47, 68, 74, 79, 80, 81, 89
Index
131
Sirius red, ix, 73, 76, 77, 81
staining, ix, 30, 56, 57, 66, 73, 76, 77, 78,
80, 81, 82, 83, 84, 87, 93, 108, 127
supportive treatment, 19
T
thioflavin T, 31, 82, 83, 89, 90
transplantation, 7, 28, 45, 67, 69
troponin(s), viii, x, 2, 7, 9, 22, 23, 91, 94,
95, 96, 101, 104
X
X-ray fibre diffraction, 84, 107