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Flashcards in Multiple Myeloma Deck (39):
1

What is multiple myeloma?

-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)

2

multiple myeloma - etiology:

-more men than women
-more AA than white
-avg age of 65

3

heavy chains are on what chromosome?

14 - IgG, IgA, IgM, IgD, IgE

4

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

IgG
IgA

5

Light chain can be?

lambda or kappa

6

MGUS vs myeloma:

-monoclonal gammopathy of und significance

-precancerous condition
-no symptoms
-1% chance per year to develop myeloma

7

symptoms of myeloma:

-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

8

criteria to diagnose multiple myeloma

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)

9

criteria to diagnose smoldering (asymptomatic) mutliple myeloma:

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

10

criteria to diagnose MGUS:

1) serum monoclonal protein <10%
3) no end organ damage related to plasma cell dyscrasia or a related b-cell lymphoprotliferative disorder

11

INternational staging system:

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

12

best survival with which stage multiple myeloma?

stage 1

13

your genetics relating to the disease determine your?

risk --> determines your treatment

depends mostly on chromosmoe 14 being involved (heavy chain)

14

treatment is based on?

RISK

15

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

at least 4 months of tx and then think transplant

16

most remission seen with what kind of transplant?

autologous!

17

Do you continue with maintenance therapy
high risk?

- YES with high risk

18

plasmacytoma what is it?

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

19

most likely area for a plasmacytoma?

humerous most
femus
pelvis
vertebra least

20

criteria for plasmacytoma

-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

extramedullary sites for plasmacytoma?
most common site?

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

22

extramedullary plasmacytomas
-location is...? good thing about this?

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

23

Diagnosis-plasmacytoma

***-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

plasmacytoma treatment:

-radiation therapy for bone or ST
-extramedullary plasmacytomas can be surgically removed

25

Plasmacytomas BONE - outcomes:
best chance of survival and/or cure is when?

-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

plasmacytomas EXTRAMEDULLARY- outcomes

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

27

amyloidosis: what is it?
how is it different?

-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

organs affected by amyloidosis:
-most common?

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

29

*What % of myeloma patients have amyloidosis?

10%

30

** Definite diagnosis for multiple myeloma?

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

Amyloidosis- Poor prognostic factors?

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

32

Most important poor prognostic factor?

cardiac - 6 months left

33

Amyloidosis treatment:
what is treatment aim?

no otpimal treatment - can use multiple myeloma drugs

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

34

Waldenstroms macroglobulinemia -what is the issue here?

-excess IgM (specific heavy change)

classified as a low grade lymphoma

-IgM in bone blood + lymphoplasmacytic lymphoma in marrow

35

Waldenstroms symptoms:

-all related to IgM - large size getting stuck somewhere

-neuropathy
-Coombs postiive autoimmune hemolytic anemia
-GI bleed
-hyperviscosity
-cytopenias
-lymphadenopathy
-hepatosplenomegaly

36

waldenstroms diagnosis:

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

-combo of lymphocytes and plasma cells

37

Waldenstroms treatment?
-asymptomatic
-symptomatic

-asym = observe
-symp = chemo, plasmapheresis (if high viscosity)

38

risk of progression of waldenstroms?

-highest in 1st 5 years

39

low grade lymphomas generally are ------?

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