Multiple Myeloma Flashcards

1
Q

What is multiple Myeloma?

A

Neoplastic disease characterized by plasma cell accumulation in the bone marrow, the presence of monoclonal protein in the serum and /or urine.

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2
Q

What is the age range of patients presenting with MM?

A

98% of cases present over the age of 40

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3
Q

What is the incidence of MM among public?

A

Is the most common primary malignancy of bone.

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4
Q

What serum finding (IL) is common among MM patients?

A

Increased IL-6 as well as IgG>IgA>IgM

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5
Q

An increased serum monoclonal IgM production would indicate?

A

Waldenstrom Macroglobulinemia.

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6
Q

What is a primary physical finding in patients with MM?

A

Punch out lesions of bone

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7
Q

How are the punch out lesions generated?

A

The neoplastic plasma cells produce osteoclast activating factor which activates the RANK receptor on Osteoclasts leading to bone destruction.

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8
Q

What do the “punch out lesions” lead to?

A

Hypercalcemia and increased risk of fracture.

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9
Q

What would plasma cells of MM cause on serum protein electrophoresis?

A

An “M spike” most commonly due to monoclonal IgG IgA

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10
Q

What pre-existing condition usually leads to MM?

A

MGUS

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11
Q

What are the tissues most likely damaged in MM?

A

CRAB

  1. Hypercalcemia
  2. Renal impairment
  3. Anemia
  4. Bone disease (Back pain)
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12
Q

What are other concurrent findings?

A
  1. Amyloid
  2. Hyperviscosity
  3. Recurrent infections (lack of Ig diversity)
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13
Q

What are the characteristic immunophenotypes of the plasma cells in MM?

A
  1. CD38

2. CD138

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14
Q

How do you obtain a difinitive diagnosis for MM?

A
  1. M-Protein in serum or urine
  2. Marrow clonal plasma cells or plasmacytoma
  3. Related organ or tissue impairment
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15
Q

Using the ISS system how is MM staged?

A

Based on B2 microglobulin and albumin levels.

  1. Stage 1 B2M <3.5g/dl
  2. Stage 3 B2M ≥5.5
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16
Q

What are the poor prognostic indicators in MM?

A
  1. Del 17p
  2. t(4;14)
  3. t(14;16)
  4. cytogenic deletion 13
  5. cytogenic hypoploidy
  6. PCL1≥3%
  7. A HIGH B2M
17
Q

What are the good prognostic indicators?

A
  1. Hyperdiploid
  2. t(11:14)
  3. t(6:14)
18
Q

What are the revolutionary drugs currently for MM?

A
  1. IMiDs

2. Proteosome Inhibitor

19
Q

How do Immunomodulatory drugs work?

A

1.Activates NK and T_Cell destruction of myeloma cells.

20
Q

What is the proteosome inhibitor used in MM?

A

Bortezomib-inhibits the the cellular proteosome and NF-KB activation, resulting in destruction of MM cells.

21
Q

What is the cut off eligibility age for MM transplants?

A

Under 70.

22
Q

What is the treatment for High risk transplant ineligible patients?

A

MP + Bortezomib

23
Q

What is the treatment for LOW risk transplant ineligible patients?

A

MP + IMiD

24
Q

What is Waldenstrom’s Macroglobulinemia?

A

Low grade lymphoma which produces excess monoclonal IgM paraprotein.

25
Q

What are symptoms of Waldenstrom’s Macroglobulinemia?

A
  1. Hyperviscosity- blurred vision, mental status change, ataxia
  2. Fatigue
  3. Weight Loss
  4. Lymphadenopathy and hepatosplenomegaly
26
Q

What treatment may work in Waldenstrom’s Macroglobulinemia that won’t in MM?

A

Rituximab due to the disease having CD20 cells.

27
Q

WHat treatment is recommended for hyperviscosity?

A

Plasmaphoresis

28
Q

What is Amyloidosis?

A

A heterogeneous group of disorders characterized by extracellular deposition of protein in an abnormal fibrillar form.

29
Q

Except for intracerebral amyloid plaques what are all amyloid deposits made of?

A

A non-fibrillary glycoprotein amylouid P which is derived from a serum precursor structurally related to C-reactive protein.

30
Q

What is the classic diagnostic histological test for Amyloidosis?

A

Staining with Congo red and viewing under polarized light.

31
Q

What is a positive Congo red test?

A

Red-Green bifringence.

32
Q

What are the different proteins involved in the different forms of Amyloidosis?

A
  1. Systemic-Ig light chains
  2. Reactive systemic AA Amyloidosis-Protein A
  3. Familial Amyloidosis-Transthyretin
  4. Localized Amyloidosis-B-Amyloid protein,peptic hormones