Myeloma

Question 1

what three tests can be used to assess immunoglobulins?

Answer 1

serum immunoglobulin levels
electrophoresis and immunofixation
serum light chain levels (urine bence jones protein)

Question 2

what does the term paraprotein mean?

Answer 2

monoclonal immunoglobulin present in urine or blood

Question 3

what part of the immunoglobulin does serum immunoglobulins levels look at?

Answer 3

Fc portion of immunoglobulin

Question 4

what is the function of electrophoresis?

Answer 4

separates protein based on charge and size

Question 5

what does serum light chain levels detect?

Answer 5

excess of light chains

Question 6

what does immunofixation do after electrophoresis?

Answer 6

determines subtype of immunoglobulin

Question 7

what are immunoglobulins produced by?

Answer 7

B cells (mostly plasma cells)

Question 8

describe the structure of immunoglobulins?

Answer 8

Y shaped

two heavy chains and two light chains

Question 9

the Fc portion is determined by what chain?

Answer 9

the heavy chain

Question 10

what is the most common immunoglobulin ?

Answer 10

IgG

- 75% of total

Question 11

what immunoglobulin is first to be produced and has the highest molecular weight?

Answer 11

IgM - pentamer

Question 12

what immunoglobulin is used in parasitic infection and hypersensitivity

Answer 12

IgE

Question 13

what are the two types of light chains?

Answer 13

kappa and lambda

Question 14

what paraproteins are present in lymphoma?

Answer 14

IgM paraproteins

Question 15

what paraproteins are present in myeloma?

Answer 15

IgG and IgA

Question 16

does lymphoma or myeloma occur in the early stages of production?

Answer 16

lymphoma since IgM is made first before IgG and IgA

Question 17

myeloma is disease of what part of the body?

Answer 17

the bone marrow

Question 18

what age is most affected by myeloma?

Answer 18

70yrs

Question 19

what is the definition of myeloma?

Answer 19

disorder of plasma cells that result in excess production of a single type of immunoglobulin

Question 20

state some features of myeloma?

Answer 20

bone disease - lytic bone lesions, cord compression and hypercalcaemia

bone marrow failure (esp anaemia)

infections

Question 21

state some systemic effects of myeloma?

Answer 21

renal failure - due to immunoglobulin deposition

hyperviscosity - causes bleeding, cardiac failure, pulmonary congestion and renal failure

hypogammaglobulinaemia

amyloidosis

Question 22

what is amyloidosis ?

Answer 22

deposition of fibrillar protein

Question 23

when amyloidosis is caused by paraprotein or light chains what is it called

Answer 23

AL amyloid

Question 24

state some features of AL amyloid?

Answer 24

nephrotic syndrome 
cardiac failure 
carpal tunnel syndrome 
autonomic neuropathy 
cutaneous infiltration

Question 25

are paraproteins common?

Answer 25

yes

Question 26

what is the condition called when an individual has a paraprotein <10% of plasma cells but has no symptoms?

Answer 26

monoclonal gammopathy of uncertain significance (MGUS)

Question 27

How is the myeloma diagnosis made?

Answer 27

>10% of plasma cells in total bone marrow cell population

Question 28

what two things is the stage of myeloma based on?

Answer 28

albumin and beta-2 micro globulin

Question 29

what is the treatment of myeloma?

Answer 29

``` chemotherapy bisphosphonate therapy RT steroids surgery - pinning of long bones and cord compression autologous stem transplant ```

Question 30

what is the medication used for bisphosphonate therapy called?

Answer 30

zoledronic acid | - blocks oestoclasts in cortical bone

Question 31

does IgM myeloma exist ?

Answer 31

no

Question 32

what is IgM paraproteins associated with ?

Answer 32

low grade lymphomas

Question 33

what is the clinical presentation of lymphoma?

Answer 33

bone marrow failure lymphadenopathy hepatosplenomegaly B symptoms

Question 34

how would a suspected IgA myeloma be confirmed ?

Answer 34

eletrophoresis and immunfixation

Question 35

what test is done for microcytic anaemia?

Answer 35

ferritin

Question 36

what does a low ferritin suggest?

Answer 36

blood loss and iron deficiency

Question 37

what investigations should be done for a low ferritin ?

Answer 37

colonoscopy and CT scan

Question 38

case - 88yo F, with fatigue, visuable disturbance and nosebleeds low Hb, normal WCC/platelets IgM paraprotein is identified on electrophoresis what is the most likely diagnosis ?

Answer 38

low grade lymphoma