5 - Plasma Cell Dyscrasias

Question 1

Multiple myeloma?

Answer 1

Malignancy of clonal plasma cells leading to monoclonal immunoglobulins
- spike of M protein/paraprotein

Question 2

What do plasma cells make?

Answer 2

IgA
IgG
IgM
IgE
IgD

Question 3

Multiple myeloma is characterized by?

Answer 3

Replacement of bone marrow

Bone destruction

Question 4

What is MGUS?

Answer 4

Monoclonial gammopathy of undetermined significance

• milder version of multiple myeloma
• be careful with it though, it could go of for like no reason

Question 5

MGUS is defined as?

Answer 5

<10% monoclonia plasma cells in bone marrow

Serum M-protein <3g/dL

No end organ damage

Question 6

MGUS previlance?

Answer 6

1% of all adults
>70=3%

Approx 1% convert to multiple myeloma. Per year

Question 7

Descrube a Multiple myeloma pt

Answer 7

Age 65
Male
Black:White 2:1

Question 8

S/s of MM?

Answer 8

Related to bony destruction

Bone pain
- back, hips, ribs
Pathologic fractures
Kidney disease
Anemia
Infection
Spinal cord compression 
Hyperviscosity syndrome

Question 9

What are hyperviscosity syndromes?

Answer 9

Mucosal bleeding
Vertigo
Nausea
Visual disturbances 
Altered consciousness

Question 10

MM PE?

Answer 10

Pallor
Bone TTP/fx
Soft tissue mass
Neurologic signs
- cord compression/neuropathy
Fever
- encapsulated organisms
Renal failure

Question 11

Why does MM cause renal failure/symptoms?

Answer 11

Hypercalcemia

Hyperuricemia

Question 12

MM labs?

Answer 12

Anemia
SPEP/UPEP w monoclonal spike
M component w sharp peak in gamma/beta globulin region
IgG (60%)
IgA (25%) light chains
Hypercalcemia - 25% 
Acute kidney injury

Question 13

MM SPEP?

Answer 13

Pic of albumin and gamma spike
- looks like vampire teeth for kids

Slide 12

Question 14

Rouleaux formation =?

Answer 14

MM

Pic on slide 13

Question 15

Imaging for MM?

Answer 15

Skeletal survey (not bone scan)
- lytic lesions

MRI or CT/PET
- extent of disease

Question 16

MM diagnosis requires?

Answer 16

All 3 of:

1. M-protein in serum/urine
2. Bone marrow aspirate >10% plasma
3. End-organ damage from plasma cells
   - min 1 crab signs

Question 17

(MM)

CRAB

Answer 17

CRAB

C - hyperCalcemia
R - Renal injury
A - anemia
B - lytic bone lesion

Question 18

What will smoldering MM look like?

Answer 18

Same as MM except:

3: NO end-organ damage from plasma cells
- 2 signs of crab

Question 19

MGUS requires what surveillance?

Answer 19

Q 3-6 mo

• SPEP/UPEP
• Hgb/Hct
• calcium
• renal panel

Bone marrow biopsy (1 x only)

Skeletal surveys (not regularly)

Question 20

MM tx?

Answer 20

Chemo +/- allogeneic stem cell transplant

Localized radiation for bone pain

tx hypercalcemia and uric acid (aggressively)

Biphosphonates decreased fractures

Question 21

Inpatient tx for MM-induced hypercalcemia?

Answer 21

1. IVF
2. furosemide (IV) (only if hypervolemic)
3. biphosphonate (IV)

Resolves hypercalcemia in >90% of cases

Question 22

MM survival?

Answer 22

Median survival of 5 yrs

Question 23

What is waldenstrom macroglobulinema?

Answer 23

B cells that are morphologically a hybrid of lymphocytes and plasma cells
- produce abnormal IgM monoclonal protein

So fucked up B cells that make bad IgM

Question 24

MM produces what kind of Ig?

Answer 24

Abnormal
IgG - 80%
IgA - 20%

Question 25

Who gets waldenstrom macroglobulinemia?

Answer 25

60-70y/o

Question 26

S/s of waldenstrom macroglobulinemia?

Answer 26

``` Fatigue Hyperviscosity Mucosal and GI bleeding AMS: lethargy, stupor, coma Cold agglutinin disease/peripheral neuropathy - 2/2 IgM paraprotein ```

Question 27

Waldenstrom macroglobulinemia PE?

Answer 27

``` Hepatosplenomegaly Lymphadenopathy Engorged retinal veins Purpura NO bone TTP ```

Question 28

Main Difference between MM and waldenstrom macroglobulinemia?

Answer 28

MM - bone pain - renal disease WM - NO bone pain - renal disease uncommon

Question 29

Labs for WM?

Answer 29

Anemia (rouleaux formation) Renal disease is uncommon

Question 30

WM diagnosis?

Answer 30

Monoclonial IgM spike on SPEP/UPEP >/= 10% of bone marrow demonstrating infiltration by small lymphocytes that exhibit plasmacytoid or plasma cell differentiation

Question 31

WM tx?

Answer 31

plasmapheresis - Hyperviscosity syndrome (tx and prophylaxis) Rituximab = anti CD20 B cell monoclonal antibody

Question 32

Refractory WM tx?

Answer 32

Autologous stem cell transplant | - relapsed/refractory pts

Question 33

Survival of WM?

Answer 33

Medial survival = 5yr | 15 yr survival = 10%