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20 Cards in this Set
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Multiple Myeloma criteria
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1. M-protein in serum and/or urine
2. Monoclonal plasma cells >10% in bone marrow 3. CRAB (at least one) - Hypercalcemia, Renal failure, Anemia, Lytic Bone lesions M-protein = abnormal immunoglobulin or fragment of it |
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What age group is primarily affected in MM?
How do you test for M-proteins? |
69 year olds (older age group)
SPEP (Serum Protein Electrophoresis) and UPEP (urine protein electrophoresis). Increased levels monoclonal antibodies (gamma globulin spike) in blood or urine. |
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What are Bence-Jones proteins?
What technique can you use to confirm the presence of M-proteins and distinguish the type of heavy/light chain (IgA, IgM, etc.)? What is the most common type seen in Multiple Myeloma? |
Bence-Jones= monoclonal light chains
Technique= Immunofixation of patient's serum. Most common = IgG. |
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If you are highly suspicious that someone has multiple myeloma but an SPEP (Serum Protein Electrophoresis) doesn't reveal an M-protein, what should you next step be?
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Look for Bence-Jones protein (examine 24 hr urine with UPEP).
Bence Jones = rapidly filtered into urine and might not show up in serum. |
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What do you expect the plasma cell count to be in this individual?
What is this cell called? |
Increase (>10% clonality). This person possibly has multiple myeloma.
Mott Cell (plasma cell with Ig globules). |
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What is this cell called? What type of Ig does it produce?
What are Russell bodies? What are Dutcher bodies? |
Flame cell (IgA)
Russell body = intra-cytoplasmic bodies Dutcher bodies= intranuclear globules |
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Describe this characteristic finding in Multiple Myeloma.
What bones are primarily affected? What lab value is notable? What type of scan is it necessary to do in patients with MM? |
Infiltration of bone by neoplastic plasma cell. Affect AXIAL skeleton most often (skull, vertebrae, pelvis).
Patients have elevated calcium from the bone destruction. This leads to neurologic sxs, weakness, confusion). Always do PLAIN FILM X-RAY! |
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Describe the mechanism of bone destruction in Multiple Myeloma.
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Myeloma cells produce RANK-L (which stimulates osteoclast differentiation). It also inhibits the production of Osteoprotegrin (OPG), which is a natural inhibitor of RANK-L.
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What does this biopsy show (consequence of Multiple Myeloma)?
What would we find in this patient's urine? |
Renal Insufficiency- free light chain proteins are toxic to tubular epithelial cells.
We would see proteinaceous casts (with Bence-Jones proteins). |
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What is shown in this blood smear?
What finding would patients present with who have this condition (hint- it's the A in CRAB)? |
Rouleaux (because Ig coating will decrease negative charge between cells that repel cells. Thus they will overlap like stacks of pennies).
Patients present commonly with anemia (normocytic, normochromic). |
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What is the ESR in patients with Multiple Myeloma?
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Sed Rate is elevated (because increase protein from the myeloma). (ESR measures how quickly RBCs fall, they fall faster in the presence of more protein).
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What is "hyperviscosity syndrome" in multiple myeloma?
Why do patients with Multiple Myeloma get recurrent bacterial infections? |
Production and aggregation of M proteins --> hyperviscosity.
Patients with multiple myeloma have suppression of normal humoral immunity (low levels of other antibodies)- this causes recurrent bacterial infections. |
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Does someone with Multiple Myeloma and elevated serum B2 microglobulin have a better or worse prognosis?
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Worse! Increased B2 microglobulin (part of MHCI) suggests worse prognosis. So do lytic lesions, high M-protein production, and high calcium.
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What is the general course of multiple myeloma (i.e. prognosis wise)?
Describe three meds that are used to treat it? |
Poor prognosis- it is progressive and NOT curable
Thalidomide (immunomodulator), Lenalidomide (related to thalidomide) + Dexamethasone, and Bortezomib (proteasome inhibitor). *also note, in the past melphalan + prednisone regimen worked well. So does autologous bone transplant. |
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What is MGUS? Where is it on the spectrum of plasma cell dyscrasias?
How do you treat it? |
MGUS= monoclonal gammopathy of undetermined significance. Simply an elevation of M-proteins (but no Bence Jones proteinuria, or elevated plasma cells in BM). No CRAB.
It is early on in spectrum (MGUS + mutations -->Smoldering Myeloma --> Multiple Myeloma). Do not treat- just watch! |
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What is "Solitary Myeloma"? What is the treatment?
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Single bone or extramedullary tumor (extramedullar has better prognosis). This is a focus of abnormal plasma cells in an area.
Treatment = XRT (because it is localized lesion). Over half progress to Multiple Myeloma. |
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What do you see in these images?
Why is it so dangerous? How is it associated with Myeloma? |
Amyloid (abnormal protein that can deposit into tissue). Beta pleated sheet = tertiary structure.
Can deposit into mutliple organs causing failure. AL = Amyloidosis caused by light chains (Ig light chains deposit as amyloid fibrils). |
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What are the characteristic findings seen here, associated with?
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Amyloidosis (this includes amyloid deposition into tongue- macroglossia), and amyloid in cutaneous blood vessels (peri-orbital prupura).
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Explain the pathogenesis of Amyloid formation in AL (Amyloidosis related to monoclonal light chains).
What special stain is shown above? |
1. Monoclonal Gammopathy causes increased mutated precursor proteins
2. These proteins are processed- degraded to yield amyloid fibrils 3 Amyloid fibrils deposited in tissue Tissue shows Green birefringence when stained with Congo red and placed under polarized light. |
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In Waldenstrom's Macroglobulinemia, what is the main monoclonal Ig to be made?
How does this contribute to the typical pathology that exists in these patients? How would you treat it? |
IgM (the large, pentameric one)
Can cause increased blood viscosity (HYPERVISCOSITY SYNDROME) causing CHF, bleeding (interference with coag pathway), raynauds, etc. Treatwith Plasmapheresis (overall, condition can be treated with chemo- Rituximab and fludarabine) |
