Plasma Cell Disorders and Myeloma

Question 1

what makes antibodies

Answer 1

plasma cells

b cells

Question 2

proteins are made up of how many heavy and light chains

Answer 2

2

2

Question 3

which antibody is a dimer

Answer 3

IgA

Question 4

which antibodies are monomers?

Answer 4

IgD
IgE
IgG

Question 5

what decides what kind of antibody an Ig is going to be?

Answer 5

depends on the recombination of the VDJ region

Question 6

once a B cell leaves a lymph node after fighting infection, what can it become?

Answer 6

a memory B cell in ciruclation OR

go back to the marrow as a plasma cell

Question 7

what do plasma cells do

Answer 7

make antibodies

Question 8

“clock face nucleus” on H and E stain

Answer 8

plasma cell

Question 9

what colour is a plasma cell’s cytoplasm and why?

Answer 9

blue as its laden with protein

Question 10

what is a paraprotein?

Answer 10

a monoclonical immunoglobulin

Question 11

paraproteins are markers of…

Answer 11

underlying clonal B cell disorder

Question 12

how is bence jones protein detected?

Answer 12

urine electrophoresis

Question 13

what is bence jones protein?

Answer 13

immunoglobulin light chains

Question 14

what is the relevance of BJP?

Answer 14

high BJP indicates an increase in light chains which = more monoclonal antibodies (myeloma) or more plasma cells

Question 15

free light chain production by plasma cells is __g per day

Answer 15

0.5

Question 16

what conditions can cause a paraproteinaemia?

Answer 16

myeloma
amyloidosis
lymphoma

Question 17

if myeloma is asymptomatic is it malignant?

Answer 17

yes it can be

Question 18

what 2 aspects of myeloma cause symptoms?

Answer 18

direct tumour cell effects

paraprotein mediated effects

Question 19

symptoms of myeloma that result from the tumour cell?

Answer 19

bone lesions
inc calcium
bone pain
marrow failure

Question 20

paraprotein symptoms of myeloma

Answer 20

renal failure
immune suppression
hyperviscosity
amyloid deposition

Question 21

most common antibody produced in myeloma

Answer 21

IgG

Question 22

“punched out, lytic lesions in the skull”…

Answer 22

myeloma

Question 23

how do light chaisn cause renal damage?

Answer 23

light chains will be filtered through the glomeruli and form casts in the PCT

Question 24

what kind of fractures are common in myeloma

Answer 24

wedge compression vertebral fractures

Question 25

what effect do myeloma cells have on osteoblasts and clasts?

Answer 25

decreased osteoblasts | inc osteoclasts

Question 26

symptoms of hypercalcaemia

Answer 26

``` bones stones moans groans thirst dehydration renal impairment ```

Question 27

Tx cast nephropathy in myeloma

Answer 27

hydration stop nephrotoxic drugs steroids/chemo to switch off light chain production

Question 28

median age of diagnosis of myeloma

Answer 28

65

Question 29

Tx myeloma

Answer 29

steroids eg dexamethasone/prednisolone cyclophosphamide thalidomide/lenalidomide high dose chemo/stem cell transplant in fit patients

Question 30

how is myeloma monitored?

Answer 30

paraprotein levels

Question 31

myeloma tends to relapse T or F

Answer 31

T

Question 32

pre-cancer form of myeloma?

Answer 32

MGUS

Question 33

symptom control of myeloma?

Answer 33

opiate analgesia radiotherapy bisphosphonates for bones vertebroplasty

Question 34

paraprotein level is

Answer 34

30

Question 35

how to tell if its MGUS or myeloma?

Answer 35

paraprotein <30g/l bone marrow plasma cells <10% asymptomatic

Question 36

Ix myeloma

Answer 36

``` BJP immunofixation calcium renal function Hb ```

Question 37

how is the presence of a paraprotein confirmed?

Answer 37

immunofixation of serum and urine

Question 38

pathophysiology of AL amyloidosis?

Answer 38

mutation in the light chain that causes amyloid to accumulate in the tissues

Question 39

Tx AL amyloidosis

Answer 39

chemo

Question 40

AL amyloidosis is a form of familial amyloidosis T or F

Answer 40

F different conditions

Question 41

symptoms of AL amyloidosis

Answer 41

nephrotic syndrome cardiomyopathy organomegaly malabsorption

Question 42

Ix AL amylodosis

Answer 42

urinalysis organ biopsy with congo red stain SAP scan to look for other organ involvement

Question 43

what is waldenstrom's macroglobulinaemia?

Answer 43

neoplasm of cells that are an intermediate between lymphocytes and plasma cells

Question 44

presence of IgM paraprotein....

Answer 44

waldenstroms macroglobulinaemia

Question 45

signs of waldenstroms

Answer 45

``` lymphadenopathy splenomegaly marrow failure hyperviscosity neuropathy ```

Question 46

symptoms of waldenstroms

Answer 46

``` fatigue visual disturbance confusion coma bleeding heart failure b symptoms eg night sweats, w loss ```

Question 47

Tx waldenstroms

Answer 47

chemo | plasmaphoresis - removing IgM