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Heme - Wier/Pallera > Plasma Cell Neoplasms > Flashcards

Plasma Cell Neoplasms Flashcards

1
Q

**What is an M spike?

A

• Sharp spike of a single type of Antibody on Serum Electrophoresis

(In a normal person you would expect to see rounded peaks)

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2
Q

**What does an IFE tell you?

A

Immunofixation Electrophoresis tells you if the antibodies being produced are kappa or lambda restricted

**Note: kappa or lambda restriction implies monoclonal proliferation

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3
Q

**Name the four components of CRAB?

A

C - Calcium
R - Renal Insufficiency
A - Anemia
B - Hypogammaglobulinemia

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4
Q

**Describe the mechanism of bone destruction in myeloma.

A
  1. Myeloma Cells Produce DKK1
  2. DKK1 stimulates STROMAL cells and OSTEOBLASTS to release RANKL and IL6
  3. RANKL and IL6 Impair osteoblast and STIMULATE OSTEOCLAST maturation
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5
Q

**What is the Durie Salmon Muliple Myeloma Staging System?

A

STAGE 1:
Hemoglobin > 10 g/dL
Calcium less than 12 mg/dL
Normal Skeletal Survey (or solidary plasmacytoma)
IgG less than 5 g/dL (IgA less than 3 g/dL)
Bence Jones Proteins less than 4 g/24 hrs

STAGE 3:
Hemoglobin less than 8 g/dL
Calcium greater than 12 mg/dL
IgG greater than 7 g/dL (IgA greater than 5g/dL)
Bence Jones Proteins greater than 12g/24hrs

***Stage 2 is in between

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6
Q

**What is the ISS staging system?

A

STAGE 1:
ß2Microglobulin and Albumin less than 3.5 g/dL
Median Survival 5 years

STAGE 2:
In between (~4 years survival)

STAGE 3:
B2Microlglobulin greater than 5.5 g/dL
Multiple Lytic Lesions
~ 2 years survival

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7
Q

**What is the worst prognostic chromosome abnormality in Myeloma

A

del17p - WORST prognostic indicator

**t(4,14) and t(14, 16) also poor prognostic indicators

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8
Q

**What two classification of drugs have changed the way we treat myeloma.
• Mechanism of Action

A

IMiDs
• Act on Immune System to Target Plasma Cells, Activate NK cells, and prevent angiogenesis

Proteosome Inhibitors:
• Block the Proteosome that targes IkB the inhibitor of NFkB, effectively inhibiting the NFkB pathway

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9
Q

**When is an autologous transplant indicated in Myeloma?

A
  • Under 70 years old

* Fairly Healthy

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10
Q

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

A

IgM (produced by this disease is much larger than IgG produced in most plasma cell lymphomas

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11
Q

**Name Three causes of Amyloidosis.

A

Light Chain Amyloid
• Secondary to Myeloma or Primary

Transthyretin Amyloidosis
• Familial

AA amyloid
• Chronic Inflammatory disorders like osteomyelitis

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12
Q

What is a hallmark of Multiple Myeloma on the Peripheral Smear?
• What causes this

A

Rouleaux

• Excess antibodies secreted by plasma cells causes platelets to stick through electrostatic forces

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13
Q

What can serum protein electrophoresis (SPEP) used for in cases of mutiple myeloma?
• What parameters can it determine?

A

SPEP:

• Look for an M-spike that can be used to 1. IDENTIFY PRESENCE OF ABNORMAL Ig 2. QUANTIFY abnormal Ig

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14
Q

What does serum and urine electrophoresis with immunofixation do that SPEP can’t do?

A

Tells you TYPE, whether its Kappa or Lambda restricted

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15
Q

What test would you do to assess the degree of cytopenia in a newly diagnosed multiple myeloma?

A

CBC with Differential

•You would expect Anemia and/or Pancytopenia because the disease is knocking out all of the bone marrow

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16
Q

Why would you expect calcium to be high in multiple Myeloma?

A

• Bone is being actively degraded by osteoclast upregulation and osteoblast down regulation

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17
Q

T or F: ß2-microglobulin is used as a prognostic factor in Multiple myeloma.

A

True

18
Q

Why would you look at a UPEP?

A

Looking for Light Chain in the urine (Bence Jones Bodies) is important because it indicates that the Kidneys may begin to fail

19
Q

How are cytogenetics and FISH important in plasma cell lymphomas (multiple myeloma)?

A

These are good indicators of the prognosis of multiple myeloma

20
Q

What criteria do you need to diagnose multiple myeloma?

A
  1. M-protein in serum or Urine
  2. Marrow Clonal plasma cells or Plasmacytoma
  3. Related organ or tissue impairment (e.g. lytic lesions)
21
Q

What are some common side effects of bone destruction secondary to Multiple Myeloma?

A
  • Pain
  • Fractures
  • Spinal Cord Compression
22
Q

What are some common side effects of Hypercalcemia secondary to Multiple Myeloma?

A
  • Altered Mental Status

* Renal Insufficiency

23
Q

Why do people with Plasma Cell Lymphoma (aka multiple myeloma) get renal insufficiency?

A
  • Light Chain Nephropathy
  • Amyloid
  • Uric Acid
  • Hypercalcemia
  • Infection
24
Q

Why would people with an increased release in antibody in mutliple myeloma be at an increased risk of infection?

A

*They are only producing monoclonal antibody while stamping out other antibody populations

25
Q

What Lymphoma is associated with amyloidosis and Hyperviscosity?

A

Multiple Myeloma

26
Q

In what population are you most likely to see MGUS?

• would you expect to see Bence Jones Bodies in the urine of these patients?

A

MGUS is most commonly seen in Old People
• 1% of people over 60
• 5% of people over 80

Bence Jones Bodies (Ig light chain) are not typically found in the urine of people with MGUS

27
Q

What is the rate of progression to malignancy in Monoclonal Gammopathy of Unspecified Significance?

A

1% per year progress to B cell malignancy

28
Q

Differentiate MGUS from Smoldering Myeloma and Multiple Myeloma?

A

(essentially) Asymptomatic Conditions = NO CRAB:
MGUS:
• M-protein in serum less than 3 mg/dL (30 g/L)
• Bone marrow clonal plasma cells less than 10%

Smoldering Myeloma:
• M-protein in serum greater than 3mg/dL (30g/L)
• Bone marrow clonal cells greater than 10%

Symptomatic (most likely) = CRAB!!
MULTIPLE MYELOMA:
• M-protein in serum or URINE
• Organ impairment, Tissue Injury (bone erosion)

29
Q

What morphologic features are important to identifying plasma cells?

A
  • Eccentric Nucleus
  • Perinuclear Clear Zone
  • ROYAL BLUE COLOR
30
Q

What are some Poor Prognostic Indicators outside of translocations for Multiple Myeloma?

A 
B2Microglobulin > 4
LDH > 190
CRP > 4
Creatinine > 2
Focal Lesions > 5
31
Q

What are some unique treatment options for Plasma Cell Lymphomas?

A
  • IMiDs - IMMUNE MODULATORY DRUGS
  • Proteosome Inhibitors
  • Bisophosphonates
32
Q

What are 2 important IMiDs?

A
  • Thalidimide

* Linalidimide

33
Q

What are 2 important proteosome inhibitors?

A
  • Bortezomib

* Carfilzomib

34
Q

What are the steps to treating someone with Multiple Myeloma that is Transplant Eligible?

A
  1. IMiD + Dex or Bortezomib + Dex
  2. Collect Stem Cells
  3. Knock down bone marrow, DO NOT USE ALKYLATING AGENTS
  4. Put cells back in
  5. Treat with IMiD+Dex
35
Q

What makes someone ineligible for Autologous transplant?

A
  • too old (greater than 70)

* Too sick

36
Q

**What is the major difference in symptoms produced by IgM Multiple Myelomas from the others?

A

HYPERVISCOSITY of blood is a big one

37
Q

You have a patient with Waldenstrom Macrolglobulinemia.

• How old do you suspect they are?

A

• At least over 40 y/o

More than 97% of cases occur in people over 40

38
Q

What sydrome is unique to people with plasma cell neoplasms that produce IgM?
• what are some symptoms

A

Hyperviscosity Syndrome

Symptoms: 
• Headache
• Ataxia
• Blurred vision
• Mental Status Change
39
Q

What therapies are most commonly used for Waldenstroms Macrogammaglobulinemia?

A
  • Purine Analogues - Fludarabine
  • Bortezomib
  • Rituximab (anti-CD20)
  • PLASMAPHERESIS with viscosity > 4
40
Q

What systems are affected by Light Chain Amyloidosis?

• What other Amyoid affects the same systems?

A

Liver
Heart
G.I.
Nervous System

*Transthyretin affects the same systems

41
Q

What systems are primarily affected by AA amyloid?

A

Renal
Hepatic
G.I.

42
Q

Where would you biopsy to detect amyloid?
• Prognosis
• Treatment?

A

Px.

1-2 years with standard treatment

Heme - Wier/Pallera (12 decks)

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