Plasma cell dysplasia

Question 1

is polyclonal rise in immunoglobulins a normal reactive response or plasma dell dysplasia

Answer 1

normal reactive response

Question 2

is monoclonal rise in immunoglobulins a normal reactive response or plasma dell dysplasia

Answer 2

plasma cell dysplasia

Question 3

monoclonal rise in immunoglobulins as a result of plasma cell dysplasia results in

Answer 3

all antibodies being identical (monoclonal)

= paraprotein formation

Question 4

does paraprotein formation come from monoclonality or polyclonality

Answer 4

monoclonality

Question 5

what happens to the kidneys when there is increased immunoglobulin production

Answer 5

cant cope = renal impairment

excrete paraproteins as bence-jones proteins

Question 6

bence jones proteins

Answer 6

myeloma

Question 7

blood film appearance of RBCs coated in proteins (ie in plasma cell dysplasia)

Answer 7

roulaeux formation (RBCs staked like a set of coins)

Question 8

differentials of plasma cell dysplasia (4)

Answer 8

myeloma
monoclonal gammopathy of undetermined significant (MGUS)
AL amyloidosis
waldenstroms macroglobulinaemia

Question 9

investigations for plasma cell dysplasia

Answer 9

electrophoresis
serum immunofixation
blood film
quantify amount of paraproteins (myeloma high, MGUS low)

Question 10

what does electrophoresis tell you in plasma cell dysplasia

Answer 10

if paraproteins are present

Question 11

what does serum immunofixation tell you in plasma cell dysplasia

Answer 11

classify type of abnormal protein (which Ig)

Question 12

what is myeloma

Answer 12

neoplastic disease of plasma cells

Question 13

what happens to plasma cells in myeloma

Answer 13

clone of faulty plasma cells = increased antibodies (monoclonal antibodies) = paraprotein formation n

Question 14

type of Ig most common in myeloma

Answer 14

IgG (can get any)

Question 15

why does myeloma present with bone pain

Answer 15

increased oestoClasts = Chew up bone

Question 16

what electrolyte imbalance occurs in myeloma and causes most of the symptoms

Answer 16

hypercalcaemia

Question 17

presentation of myeloma (5)

Answer 17

bone 
kidney stones 
psychiatric moans 
abdo groans 
thirst/dehydration

Question 18

bone pain
‘punched out’ lytic lesions
‘pepper pot’ skull
pathological fractures (from weakened bone)

Answer 18

myeloma

Question 19

investigations for myeloma

Answer 19

electrophoresis
ESR
skeletal survey - to see which bones affected

Question 20

ESR in myeloma

Answer 20

raised

Question 21

chronic management of myeloma

Answer 21

chemo + steroids/bisphosphonates/thalidomide

Question 22

symptomatic management of myeloma

Answer 22

analgesia (NOT NSAIDs bc renal impairment) for pain

bisphosphonates - for bone pain and hypercalcaemia

Question 23

prognosis of myeloma

is it curable

Answer 23

not curable

aim for remission, will probs relapse

Question 24

most common cause of paraproteinemia (presence of paraproteins)

Answer 24

monoclonal gammopathy of undetermined significance (MGUS)

Question 25

what is monoclonal gammopathy of undetermined significance (MGUS)

Answer 25

when paraproteins are present but at v low concs = asymptomatic, doesn't cause harm

Question 26

who likely has asymptomatic monoclonal gammopathy of undetermined significance (MGUS)

Answer 26

old people! so don't worry if you find it

Question 27

does monoclonal gammopathy of undetermined significance (MGUS) lead to myeloma/waldenstoms

Answer 27

v v rarely

Question 28

what happens to the paraproteins in AL amyloidosis

Answer 28

they clump together = amyloid formation = accumulation in tissues = serious organ damage

Question 29

investigations for AL amyloidosis

Answer 29

congo red stain - 'apple green bifringence' under Polarised light urinalysis - for proteinuria EHCO - to check heart LFTs - to check liver

Question 30

congo red stain - 'apple green bifringence'

Answer 30

amyloidosis

Question 31

management of AL amyloidosis | how does it work

Answer 31

chemo switches off light chain production = no more amyloid production = no further organ damage (can undo whats been done though)

Question 32

what type of cancer is waldenstroms macroglobulinaemia

Answer 32

non hodgkins lymphoma

Question 33

what type of Ig paraprotein is in waldenstroms

Answer 33

IgM lol only thing you know but give yourself a pat on the back

Question 34

what is significant about the IgM paraprotein in waldenstroms macroglobulinaemia what does this cause

Answer 34

IgM is a tetramer = big protein = MACROglobulinaemia cause hyperviscosity bc so big!

Question 35

presentation of hyperviscosity in waldenstroms macroglobulinaemia

Answer 35

bleeding fatigue coma confusion

Question 36

presentation of waldenstroms macroglobulinaemia

Answer 36

like lymphoma! - night sweats, weight loss, fatigue

Question 37

investigations for waldenstorms macroglobulinaemia

Answer 37

increased viscosity | protein electrophoresis - to see IgM paraproteins

Question 38

management of waldenstroms macroglobulinaemia (3)

Answer 38

plasmapheresis - remove and replace patient plasma with non IgM rich plasma chemo - long term proteasome inhibitors - causes apoptosis of proteins