5 - Plasma Cell Dyscrasias Flashcards

1
Q

Multiple myeloma?

A

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

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2
Q

What do plasma cells make?

A
IgA
IgG
IgM
IgE
IgD
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3
Q

Multiple myeloma is characterized by?

A

Replacement of bone marrow

Bone destruction

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4
Q

What is MGUS?

A

Monoclonial gammopathy of undetermined significance

  • milder version of multiple myeloma
  • be careful with it though, it could go of for like no reason
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5
Q

MGUS is defined as?

A

<10% monoclonia plasma cells in bone marrow

Serum M-protein <3g/dL

No end organ damage

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6
Q

MGUS previlance?

A

1% of all adults
>70=3%

Approx 1% convert to multiple myeloma. Per year

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7
Q

Descrube a Multiple myeloma pt

A

Age 65
Male
Black:White 2:1

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8
Q

S/s of MM?

A

Related to bony destruction

Bone pain
- back, hips, ribs
Pathologic fractures
Kidney disease
Anemia
Infection
Spinal cord compression 
Hyperviscosity syndrome
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9
Q

What are hyperviscosity syndromes?

A
Mucosal bleeding
Vertigo
Nausea
Visual disturbances 
Altered consciousness
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10
Q

MM PE?

A
Pallor
Bone TTP/fx
Soft tissue mass
Neurologic signs
- cord compression/neuropathy
Fever
- encapsulated organisms
Renal failure
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11
Q

Why does MM cause renal failure/symptoms?

A

Hypercalcemia

Hyperuricemia

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12
Q

MM labs?

A
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
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13
Q

MM SPEP?

A

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

Slide 12

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14
Q

Rouleaux formation =?

A

MM

Pic on slide 13

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15
Q

Imaging for MM?

A
Skeletal survey (not bone scan)
- lytic lesions

MRI or CT/PET
- extent of disease

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16
Q

MM diagnosis requires?

A

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
17
Q

(MM)

CRAB

A

CRAB

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

18
Q

What will smoldering MM look like?

A

Same as MM except:

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

19
Q

MGUS requires what surveillance?

A

Q 3-6 mo

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

Bone marrow biopsy (1 x only)

Skeletal surveys (not regularly)

20
Q

MM tx?

A

Chemo +/- allogeneic stem cell transplant

Localized radiation for bone pain

tx hypercalcemia and uric acid (aggressively)

Biphosphonates decreased fractures

21
Q

Inpatient tx for MM-induced hypercalcemia?

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

Resolves hypercalcemia in >90% of cases

22
Q

MM survival?

A

Median survival of 5 yrs

23
Q

What is waldenstrom macroglobulinema?

A

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

24
Q

MM produces what kind of Ig?

A

Abnormal
IgG - 80%
IgA - 20%

25
Q

Who gets waldenstrom macroglobulinemia?

A

60-70y/o

26
Q

S/s of waldenstrom macroglobulinemia?

A
Fatigue
Hyperviscosity
Mucosal and GI bleeding
AMS: lethargy, stupor, coma
Cold agglutinin disease/peripheral neuropathy
- 2/2 IgM paraprotein
27
Q

Waldenstrom macroglobulinemia PE?

A
Hepatosplenomegaly
Lymphadenopathy
Engorged retinal veins
Purpura
NO bone TTP
28
Q

Main Difference between MM and waldenstrom macroglobulinemia?

A

MM

  • bone pain
  • renal disease

WM

  • NO bone pain
  • renal disease uncommon
29
Q

Labs for WM?

A

Anemia (rouleaux formation)

Renal disease is uncommon

30
Q

WM diagnosis?

A

Monoclonial IgM spike on SPEP/UPEP

> /= 10% of bone marrow demonstrating infiltration by small lymphocytes that exhibit plasmacytoid or plasma cell differentiation

31
Q

WM tx?

A

plasmapheresis
- Hyperviscosity syndrome (tx and prophylaxis)

Rituximab
= anti CD20 B cell monoclonal antibody

32
Q

Refractory WM tx?

A

Autologous stem cell transplant

- relapsed/refractory pts

33
Q

Survival of WM?

A

Medial survival = 5yr

15 yr survival = 10%