Myeloma and Plasma Cell Dyscrasias

Question 1

What are immunoglobulins composed of?

Answer 1

2 heavy chains and 2 light chains

Question 2

Which immunoglobulins ae monomers?

Answer 2

IgD
IgE
IgG

Question 3

Which immunoglobulins are dimers?

Answer 3

IgA

Question 4

Which immunoglobulins are pentamers?

Answer 4

IgM

Question 5

Where are B cells produced?

Answer 5

bone marrow

Question 6

What becomes the antigen binding site on the immunoglobulin?

Answer 6

Random selection of V D and J genes

Question 7

Where do B cells travel to get antigens?

Answer 7

Follicle germinal centre of the lymph node

Question 8

What is found in the pale perinuclear area of a plasma cell?

Answer 8

Golgi apparatus

Question 9

Describe the nucleus of a plasma cell

Answer 9

Clock-face

Question 10

Which scenarios would cause a polyclonal increase in immunoglobulins?

Answer 10

Infection
Autoimmunity
Malignancy
Liver disease

Question 11

Give examples of causes of paraproteinaemia?

Answer 11

MGUS
Amyloidosis 
Myeloma
Lymphoma
CLL

Question 12

How are immunoglobulins detected in the blood?

Answer 12

Electrophoresis

Immunofixation

Question 13

What is a Bence-Jones protein?

Answer 13

Kappa free immunoglobulin light chain

Question 14

Where are Bence-Jones proteins found in myeloma?

Answer 14

Urine

Question 15

Outline the pathogenesis in multiple myeloma

Answer 15

Uncontrolled overproduction of one immunoglobulin which pushes out other healthy cells from the bone marrow

Question 16

What is a paraprotein?

Answer 16

Abnormal immunoglobulin light chain produced by abnormal immunoglobulin

Question 17

Why are lytic lesions found in myeloma?

Answer 17

Abnormal immunoglobulins activate osteoclasts through IL-6

Question 18

What is the most common myeloma?

Answer 18

IgG

Question 19

What is the second most common myeloma?

Answer 19

IgA

Question 20

How does myeloma present?

Answer 20

HyperCalcaemia
Renal failure
Anaemia
Bone pain
(CRAB)

Question 21

How does the myeloma-induced renal failure present?

Answer 21

N%V
Pruritus and muscle cramps
Dyspnoea and oedema

Question 22

What causes nausea and vomiting in renal failure?

Answer 22

Uraemia

Question 23

What causes pruritus and muscle cramps in renal failure?

Answer 23

Increased phosphate

Question 24

What causes dyspnoea and oedema in renal failure?

Answer 24

Fluid retention

Question 25

Describe the anaemia in myeloma?

Answer 25

Normocytic normochromic

Question 26

Describe the bone pain in myeloma

Answer 26

Worse on activity | Common in spine and ribs

Question 27

What causes confusion, weakness and fatigue in myeloma?

Answer 27

Hypercalcaemia

Question 28

What causes headache and visual disturbance in myeloma?

Answer 28

Hyper viscosity

Question 29

What causes peripheral neuropathy in myeloma?

Answer 29

Amyloid deposition

Question 30

What do U&Es show in myeloma?

Answer 30

Raised CK | Hypercalcaemia

Question 31

What does blood film show in myeloma?

Answer 31

Rouleaux

Question 32

How is myeloma status assessed?

Answer 32

Bone marrow biopsy Immunohistochemistry Cytogenetics Skeletal survey (MRI)

Question 33

What is the criteria for symptomatic myeloma?

Answer 33

Clonal plasma cells >10% of biopsy Paraprotein in serum or urine End-organ damage

Question 34

What is the criteria for asymptomatic myeloma?

Answer 34

Clonal plasma cells >10% biopsy Serum paraprotein >30g/L No myeloma related end organ damage

Question 35

How is asymptomatic myeloma treated?

Answer 35

Watchful waiting

Question 36

What is the MGUS criteria?

Answer 36

Serum paraprotein <30g/l Clonal plasma cells <10% on bone marrow biopsy No myeloma end organ damage

Question 37

How is myeloma treated?

Answer 37

<65 - combination chemo and autologous stem cell transplant | >65 - combination chemo

Question 38

What is "maintenance treatment" for myeloma?

Answer 38

Low dose chemo on a regular basis

Question 39

What symptomatic relief is given in myeloma?

Answer 39

Analgesia - not NSAID Local radio - for cord comp Bisphosphonates - bone pain Vertebroplasty - stabilise vertebra

Question 40

What drugs are used with chemo in myeloma treatment?

Answer 40

Corticosteroids, dexmet, prednisolone Alkylating agents Thalidomide Lenalidomide

Question 41

Give examples on an alkylating agent

Answer 41

Cylcophosphamide | Melphalan

Question 42

What is AL amyloidosis?

Answer 42

Mutation in the light chain of antibodies so they can't be broken down Accumulate in tissue as insoluble beta pleated sheet

Question 43

How is AL amyloidosis treated?

Answer 43

Chemo to switch off light chain supply

Question 44

What does biopsy and stain show in AL amyloidosis?

Answer 44

Congo red stain | Apple-green birefringence under polarised light

Question 45

Which investigations can be used to show amyloid deposition in other organs?

Answer 45

Serum amyloid scintigraphy scan Echo Nephrotic range proteinuria

Question 46

Which immunoglobulin is involved in Waldenstrom's macroglobulinaemia?

Answer 46

IgM

Question 47

What is Waldenstrom's Macroglobulinaemia?

Answer 47

Clonal disorder of cells resulting in a lymphoplasmacytoid neoplasm

Question 48

Why does Waldenstrom's Macroglobulinaemia cause a greater degree of hyperviscosity than myeloma?

Answer 48

IgM has more binding sites

Question 49

How does hyperviscosity syndrome present?

Answer 49

``` Fatigue Visual problems Confusion/coma Bleeding Cardaic failure ```

Question 50

How is Waldenstrom's Macroglobulinaemia treated?

Answer 50

Plasmapheresis to physically remove IgM | Chemo