3.27 WIER Multiple Myeloma Flashcards Preview

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Flashcards in 3.27 WIER Multiple Myeloma Deck (13):
1

Mult Myeloma

Malignancy of late B cels that mature principally into neoplastic plasma cells that generally produce a complete and/or partial (light chain) monoclonal Ig protein

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2

Name the four components of CRAB

CRAB:

-HyperCalcemia: altered mental status, renal insufficiency

-Renal Insufficiency: light chan nephropathy (and tubules), amyloid, uric acid, hypercalcemia, infection

-Anemia

-Bone Destruction: pain, fractures, spinal cord compression

-CRAB has to be present for the diagnosis of a multiple myeloma

Others: hypogammaglobulinemia, amyloidosis, hyperviscosity

 

 

 

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3

What is an M spike?

-monoclonal protein in the serum or urine, from overproduction of one specific anti-body produced by the malignant plasma cell

-Will cause Rouleaux formation of the RBCs (line of coins)

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4

What does an IFE tell you?

Immuno-Fixation Electrophoresis

-It will tell you what kind of anti-body it is (heavy and light chain)

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5

Describe the mechanism of bone destruction in myeloma.

-myeloma cells produce DKK1, leading to increased RANKL and IL6 by osteoblast progenitor cells and marrow stromal cells

-Osteoblast differentiation is blocked and osteoclast muturation is stimulated

-Osteoclasts break down bone

6

Name the worst prognostic chromosome abnormality in Myeloma.

-People with Del 17p do the worst

-t(4;14) and t(14;16) are also high risk

7

Stage a myeloma with Durie Salmon and ISS.

  • Durie-Salmon:
  • Stage 1: Hgb>10g/dl, Ca normal, Normal skeletal survey or solitary plasmacytoma, low M protein w/ IgG<5 or IgA<3, Bence Jones <4
  • Stage 2: Neither 1 or 3
  • Stage 3 (one or more of the following): Hgb<8.5, Ca>12, High M component w/ IgG>7 or IgA.5, Bence Jones>12g/24hr, multiple lytic lesions
  • ISS STAGING
  • Stage 1: beta2 Mycroglobulin <3.5 and albumin >3.5 (survival median of 62 months)
  • Stage 2: Beta2M <3.5 and albumin<3.5 or beta2M 3.5 to 5.5 (median survival of 44 months)
  • Stage 3: B2M >5.5 (median survival 29 months)

8

When is an autologous transplant indicated in myeloma?

-Generally eligable if pt is under 70 and in relatively good health (will have better chance to survive transplant) 

-Allows high doses of chemo therapy (dose lim tox: BM suppression) and then give stem cells back to patient

9

What two classifications of new drugs have changed the way we treat myeloma? Mechanism of action?

-IMiDs (immune modulatory drugs): ex is thalidomide and linolidomide, cause activation of T Cells, cause destruction of myeloma cells, alter stromal structure (VEGF decrease and IL-6). Have extracellular and intercellular activities on the myeloma cells. Very effective drugs for myeloma with minimal toxicity

-Proteosome Inhibitors: ex Bortezomib, inhibit protein destruction which leads to destruction of the myeloma cells

10

Why do Waldenstrom patients become hyperviscous more commonly than myeloma patients?

-Walkdenstrom's is b/t mult. myeloma and CLL/SLL and considered a low grade lymphoma which produces excess IgM paraprotein

-IgM is a bigger molecule  so will cause hyperviscosity (serum viscosity >4) faster than overproduction of other abs

treat with Rituximab and Bortezumab

-Not a curable disease only treat if symptoms present

-Can only improve survival by treating patient when they are symptomatic, treating when asymptomatic will not prolong survival

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11

Name three causes of amyloidosis

(1) Light Chain Amyloidosis: can be primary or secondary to myeloma, involves nervous system, Heart, GI and liver

(2) Transthyretin: Familial, same systemic involvment as light chain, cause by enzyme defect

(3) Amyloidosis AA: due to chronic inflamm disorders, renal disease, hepatic and GI involvement

Prognosis: 1-2 yrs with standard treatment, possibly longer with transplant

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12

Monoclonal gammaopathy of Undetermined significance (MGUS)

>Precursor to Mult Myeloma, more common also

>Less than 10% plasma cells in the bone marrow

>Rate of progression to mult is 1%/yr

-Other Igs usually normal

-Increase in protein suggest progression towards malignancy

-Less likely to have Bence Jones proteinuria

absense of CRAB (so cant be myeloma)

13

Smoldering Myeloma

Serum monoclonal protein > 3gm/dl and/or BM plasma cells >/= 10%

-NO CRAB***

-Do not treat, it wont help, can treat symptoms