Myeloma and Paraproteins

Question 1

Antibodies are produced by which type of cell?

Answer 1

B cell - plasma cell

Question 2

Function of antibodies

Answer 2

Recognise and bind pathogens

Question 3

How many heavy chains does an antibody have?

Answer 3

2

Question 4

How many types of heavy chains are there?

Answer 4

5 - IgG, IgM, IgA, IgD, IgE

Question 5

How many light chains does an antibody have?

Answer 5

2

Question 6

Which antibody heavy chain exists as a pentamer?

Answer 6

IgM

Question 7

Which antibody heavy chain has the highest molecular weight?

Answer 7

IgM

Question 8

Which antibody heavy chain is involved in the initial stage of antibody production?

Answer 8

IgM

Question 9

Which heavy chain is the most prevalent antibody subclass?

Answer 9

IgG

Question 10

Which heavy chain is invovled in mucous membrane immunity?

Answer 10

IgA

Question 11

IgE heavy chain antibodies are involved in which immune responses?

Answer 11

Parasite immune responses

Hypersensitivity

Question 12

Name 2 types of light chain

Answer 12

Kappa or Lambda

Question 13

How are light chains selected for each cell?

Answer 13

Random

Question 14

How many types of light chains will antibodies have produced by one cell>

Answer 14

One cell produces one specific light chain with one specificity

Question 15

Which type of antibody chain can be found free and at low levels in the blood?

Answer 15

Light chain

Question 16

What does immunoglobulin total levels indicate?

Answer 16

The number of immunoglobulin subclasses

Question 17

What is a paraprotein?

Answer 17

Identical antibodies, produced via a clonal process
Monoclonal immunoglobulin present in blood or urine //CSF
Monoclonal proliferation of B cell/plasma cell somewhere in the body

Question 18

What can be used to diagnose presence of paraproteins?

Answer 18

Serum Protein electrophoresis

Question 19

How does serum protein electrophoresis identify paraproteins?

Answer 19

Separates proteins according to size and charge to illicit a band pattern

Question 20

Other tests used to assess paraproteins

Answer 20

Immunofixation - identifies class
Light chains - imbalance/excess of light chains in urine/serum

Question 21

IgM paraproteins are associated with which disease?

Answer 21

Lymphoma

Question 22

Lymphoma can be characterised by presence of which type of paraprotein?

Answer 22

IgM

Question 23

IgM antibodies are produced by which cells and which stage of immune response?

Answer 23

Maturing B lymphocytes at beginning of immune response

Question 24

Myeloma is associated with which paraproteins?

Answer 24

IgA

IgG

Question 25

IgA and IgG paraproteins are produced by which cells

Answer 25

Plasma cells after isotype switching

Question 26

Myeloma is a neoplastic disorder of which type of cell?

Answer 26

Plasma cell

Question 27

Myeloma occurs in which 2 groups of the population>

Answer 27

Those in 7th decade | Black population

Question 28

Clinical features of myeloma

Answer 28

Bone Disease Bone Marrow Failure Infections CRAB- hypercalcaemia, renal disease, anaemia, bone disease

Question 29

Describe the 4 features of bone disease found in cases of myeloma

Answer 29

Lytic lesions - salt pepper skull Pathological fractures Cord compression Hypercalcaemia

Question 30

Describe the appearance of the skull in case of myeloma

Answer 30

Salt pepper skull | Lytic lesions

Question 31

How does renal failure occur due to paraproteins?

Answer 31

Cast nephropathy | IgG deposits- blocks renal tubules

Question 32

Four Effects of paraproteins

Answer 32

Renal Failure Hyperviscosity Hypogammaglobulinaemia Amyloidosis

Question 33

Hyperviscosity causes bleeding in which four tissues in particular?

Answer 33

Retinal, oral, nasal and cutaneous

Question 34

4 Clinical consequences of hyperviscosity

Answer 34

Renal failure Cardiac failure Pulmonary congestion Confusion

Question 35

Hypogammaglobulinaemia has which clinical consequence

Answer 35

Tendency for infection

Question 36

Which type of protein forms deposits in amyloidosis

Answer 36

Fibrillar protein

Question 37

Amyloidosis is given which name when caused by paraproteins or light chains

Answer 37

Amyloid Light-chain Amyloidosis (AL) OR Primary amyloidosis

Question 38

Amyloidosis is a feature of which 5 conditions

Answer 38

``` Nephrotic syndrome Cardiac Failure - LVH Carpal tunnel syndrome Autonomic neuropathy Cutaneous infiltration ```

Question 39

Paraprotein can trigger inflammation leading to which condition

Answer 39

Amyloidosis

Question 40

Amyloidosis is typically triggered by which type of conditions

Answer 40

Inflammatory - RA, TB

Question 41

Paraproteins are common and can lead to which diagnosis

Answer 41

Monoclonal Gammopathy of Uncertain Significance (MGUS)

Question 42

Criteria for Myeloma diagnosis

Answer 42

Excess plasma cells in bone marrow | >10% of population of cells

Question 43

Criteria for Asymptomatic Myeloma diagnosis

Answer 43

Paraprotein + >10% cells | No CRAB symptoms

Question 44

What are CRAB symptoms for Myeloma?

Answer 44

Hypercalcaemia Renal disease Anaemia Bone disease

Question 45

Most common type of paraprotein (55%)

Answer 45

IgG Followed by IgA, Light chain only

Question 46

Staging of paraprotein is based on?

Answer 46

Albumin and beta 2 microglobulin

Question 47

Treatment of Myeloma

Answer 47

``` Chemotherapy Radiotherapy Bisphosphonate therapy - zoledronic acid Steroids Surgery Autologous stem cell transplant ```

Question 48

What is used in chemotherapy in the treatment of myeloma?

Answer 48

Proteasome inhibitors - carfilzomib, bortezomib IMiDs (lenalidomide, pomalidomide) Monoclonal antibodies

Question 49

What surgery is involved in treatment of myeloma?

Answer 49

Pinning of long bones | Decompression of the spinal cord

Question 50

Clinical presentation of lymphoma

Answer 50

``` Lymphadenopathy Bone marrow failure Hepatosplenomegaly B symptoms Paraprotein related symptoms ```

Question 51

What are B symptoms?

Answer 51

Fever, weight loss, fatigue, pruritus, drenching night sweats

Question 52

What symptoms are rare with lymphomas?

Answer 52

Bone disease