17 - Plasma Cell Dyscrasias

Question 1

back pain + anemia + rouleaux on PBS

Answer 1

multiple myeloma

Question 2

multiple myeloma on xray

Answer 2

multiple lytic lesions

Question 3

dx of multiple myeloma

Answer 3

proliferation of malginant plasma cells
secretion of monoclonal immunoglobulin protein
typically multiple foci of plasma cells in bone marrow
might see some binucleate plasma cells

Question 4

epi for multiple myeloma

Answer 4

old (only 2% <40 yo)

blacks more often than whites

Question 5

3 proteins in multiple myeloma resulting in lytic bone dz and hypercalcemia

Answer 5

inc MIP-1alpha (macrophage inflammatory protein) - inc osteoclast activity
inc RANKL - inc osteoclast activity
dec OPG (osteoprotegerin) - decoy receptor for RANKL

Question 6

major treatment option for bone related sx of multiple myeloma

Answer 6

bisphosphonates

Question 7

how does multiple myeloma cause anemia?

Answer 7

IL6 inc > stim production of hepcidin > dec release of iron from macrophages > anemia, possibly B12 deficiency

Question 8

4 problems common in multiple myeloma

Answer 8

CRAB:
calcemia (hyper)
renal insufficiency
anemia
bones - lytic lesions

Question 9

unique part of diagnostic workup for multiple myeloma

Answer 9

urine protein electrophoresis to look for light chains

Question 10

staging of multiple myeloma

Answer 10

1 - albumin > 3.5, b2-microglob 5.5

Question 11

tx for multiple myeloma

Answer 11

not a candidate for auto-BMT - MP +/- thalidomide

candidate - dex +/- (lenalidomide, bortezomib, or both)

Question 12

monoclonal gammopathy of undertermined significance (MGUS)

Answer 12

basically subclinical form of multiple myeloma
monoclonal IG < 3 g/dL
under 10% plasma cells in bone marrow
no bone lesions or sx
normal levels of IGs

Question 13

rate of transformation from MGUS to multiple myeloma

Answer 13

~1% / yr

Question 14

Mayo’s M-spikes most often indicate which conditions

Answer 14

MGUS, multiple myeloma, amyloidosis are most likely diagnosis

Question 15

amyloidosis pathogenesis

Answer 15

fibrils formed by low MW protein subunits (light chains) > deposit in organs > irreversible dysfunction

Question 16

difference between primary and secondary amyloidosis

Answer 16

primary (AL)- due to deposition of fibrils made of light chains
secondary (AA) - fibrils made of other proteins

Question 17

amyloidosis clinical presentation

Answer 17

cardiomyopathy, hepatomegaly, nephrotic syndrome, neuropathy, carpal tunnel syndrome, periorbital purpura, waxy skin w/ easy bruising, enlarged muscles (tongue, deltoids), carpal tunnel, impaired coagulation, fatigue

Question 18

dx of amyloidosis

Answer 18

amyloid has apple green birefringence w/ Congo red stain

biopsy affected tissue and stain it

Question 19

tx of primary amyloidosis

Answer 19

melphalan and prednisone

bortezomib

Question 20

lymphoplasmacytic lymphoma (waldenstrom’s macroglobulinemia)

Answer 20

uncommon low grade malignancy
malignant plasmacytoid lymphocytes make IgM
earlier stage malignant cell than in multiple myeloma

Question 21

presentation of waldenstrom’s macroglobulinemia

Answer 21

LAN and splenomegaly, hyperviscosity, cryoglobulinemia, neuropathy
lots of nonspecific sx - weakness, fatigue, wt loss, epistaxis, gingival bleeding, anemia, fever, night sweats

Question 22

hyperviscosity syndrome

Answer 22

blurring or loss of vision
HA, vertigo, nystagmus, dizziness
sudden deafness, diplopia or ataxia
mental confusion
disturbances of consciousness, stroke, coma

Question 23

tx of waldenstroms macroglobulinemia

Answer 23

plasmapheresis if hyperviscosity

other sx: chemo or biologics