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Heme Exam 3 > Multiple Myeloma > Flashcards

Multiple Myeloma Flashcards

1
Q

What is multiple myeloma?

A
  • cancer of plasma cells
  • plasma cells typically produce antibodies and pay key role in immune immune function
  • overgrowth of abnormal plasma cells –> m-protein (MONOCLONAL)
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2
Q

multiple myeloma - etiology:

A
  • more men than women
  • more AA than white
  • avg age of 65
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3
Q

heavy chains are on what chromosome?

A

14 - IgG, IgA, IgM, IgD, IgE

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4
Q

which heavy chain will you see most often? second most often?

A

IgG

IgA

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5
Q

Light chain can be?

A

lambda or kappa

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6
Q

MGUS vs myeloma:

A

-monoclonal gammopathy of und significance

  • precancerous condition
  • no symptoms
  • 1% chance per year to develop myeloma
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7
Q

symptoms of myeloma:

A
  • bone pain/fractures (lytic)
  • numbness/tingling in lower back/legs
  • weight loss
  • weakness (anemia), N/V, altered mental status (Could be hypercalcemia)
  • short of breath
  • urinary issues
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8
Q

criteria to diagnose multiple myeloma

A

1) presence of a serum or urinary monoclonal protein (some combo of heavy and light chain)
2) presence of clonal plasma cells in the bone marrow or a plasmacytoma
3) presence of end organ damage felt related to the plasma cell dyscrasia (such as increases Ca, lytic bone lesions, anemia, or renal failure)

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9
Q

criteria to diagnose smoldering (asymptomatic) mutliple myeloma:

A

1) serum monoclonal proteins >=3g/dL and or >=10% to <60% of bone marrow clonal plasma cells
2) no end organ damage related to plasma cell dyscrasia

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10
Q

criteria to diagnose MGUS:

A

1) serum monoclonal protein <10%

3) no end organ damage related to plasma cell dyscrasia or a related b-cell lymphoprotliferative disorder

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11
Q

INternational staging system:

A

stage 1: Beta2M=3.5
stage 2: neither stage 1 or 3
stage 3: B2M>=5.5

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12
Q

best survival with which stage multiple myeloma?

A

stage 1

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13
Q

your genetics relating to the disease determine your?

A

risk –> determines your treatment

depends mostly on chromosmoe 14 being involved (heavy chain)

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14
Q

treatment is based on?

A

RISK

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15
Q

high risk patient,w hat is important part with treatment?

A

at least 4 months of tx and then think transplant

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16
Q

most remission seen with what kind of transplant?

A

autologous!

17
Q

Do you continue with maintenance therapy

high risk?

A
  • YES with high risk
18
Q

plasmacytoma what is it?

A

focused area of plasma cells in the bone or other area (extramedullary)

19
Q

most likely area for a plasmacytoma?

A

humerous most
femus
pelvis
vertebra least

20
Q

criteria for plasmacytoma

A
  • all patients will have either no or low levels of M-protein in serum or urine
  • serum Ig levels are usually preserved
  • serum free light chains may be elevated
21
Q

extramedullary sites for plasmacytoma?

most common site?

A 
-URT - MOST COMMON
GI,
CNS
bladder
thyroid
breasts
testes
parathyroid
LN
22
Q

extramedullary plasmacytomas

-location is…? good thing about this?

A

more localized and more curable with localized radiotherapy and/or can be removed

23
Q

Diagnosis-plasmacytoma

A
  • **-serum or urine M protein can be present in low level
  • biopsy proven solitary lesion of bone or soft tissue with evidence of clonal plasma cells
  • normal skeletal survey and MRI of spine and pelvis
  • absence of end organ damage that can be attributed to a plasa cell disorder
  • bone marrow with no evidence of plasma cells

-SHOULD NOT HAVE END ORGAN DAMAGE

24
Q

plasmacytoma treatment:

A
  • radiation therapy for bone or ST

- extramedullary plasmacytomas can be surgically removed

25
Q

Plasmacytomas BONE - outcomes:

best chance of survival and/or cure is when?

A
  • best chance is when M-protein disapears and no evidence of active disease
  • majority of patients progress to myeloma within 2 years
  • 20% disease free at 10 years
26
Q

plasmacytomas EXTRAMEDULLARY- outcomes

A

-approx 70% of patients will be alive and disease free at 10 years

27
Q

amyloidosis: what is it?

how is it different?

A
  • protein formed but it has an abnormal conformation
  • protein conformation disorder associated with clonal plasma cell dyscrasia
  • extracellular deposition of light chain fragments in an abnormal insoluble fibrillar form
  • poor correlation between impairment of organ function
28
Q

organs affected by amyloidosis:

-most common?

A
  • kidney most common - nephrotic syndrome
  • heart -restrictive cardiomyopathy
  • nerves-peripheral neuropathy
  • -liver-hepatomegaly
  • tongue-macroglossia
  • purpura (PERIORBITAL) RACCOON EYES
29
Q

*What % of myeloma patients have amyloidosis?

A

10%

30
Q

** Definite diagnosis for multiple myeloma?

A

CONGO RED STAIN - red-green birefringence under polarized light

NOT PERFECT THOUGH 50% sensitive with BM, 70% with fat pad

more sentitive with multiorgan involvement

31
Q

Amyloidosis- Poor prognostic factors?

A
  • CARDIAC (6 mo left)
  • autonomic neuropathy
  • liver involvement with hyperbilirubinemia
  • lack of suppression of underlying clonal disease by chemo
  • associated multiple myeloma
32
Q

Most important poor prognostic factor?

A

cardiac - 6 months left

33
Q

Amyloidosis treatment:

what is treatment aim?

A

no otpimal treatment - can use multiple myeloma drugs

-aim is to retard further deposition since deposition is NOT reversible

34
Q

Waldenstroms macroglobulinemia -what is the issue here?

A

-excess IgM (specific heavy change)

classified as a low grade lymphoma

-IgM in bone blood + lymphoplasmacytic lymphoma in marrow

35
Q

Waldenstroms symptoms:

A

-all related to IgM - large size getting stuck somewhere

  • neuropathy
  • Coombs postiive autoimmune hemolytic anemia
  • GI bleed
  • hyperviscosity
  • cytopenias
  • lymphadenopathy
  • hepatosplenomegaly
36
Q

waldenstroms diagnosis:

A
  • any size of IgM monoclonal gammopathy
  • bone marrow > 10% monoclonal lymphocytes and plasma cell differentiation

-combo of lymphocytes and plasma cells

37
Q

Waldenstroms treatment?

  • asymptomatic
  • symptomatic
A
  • asym = observe

- symp = chemo, plasmapheresis (if high viscosity)

38
Q

risk of progression of waldenstroms?

A

-highest in 1st 5 years

39
Q

low grade lymphomas generally are ——?

A

NOT curable - you just livewith it and treat symptoms when they bug you

Heme Exam 3 (8 decks)

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