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J Haem > Plasma cell dysplasia > Flashcards

Plasma cell dysplasia Flashcards

1
Q

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

A

normal reactive response

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2
Q

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

A

plasma cell dysplasia

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3
Q

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

A

all antibodies being identical (monoclonal)

= paraprotein formation

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4
Q

does paraprotein formation come from monoclonality or polyclonality

A

monoclonality

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5
Q

what happens to the kidneys when there is increased immunoglobulin production

A

cant cope = renal impairment

excrete paraproteins as bence-jones proteins

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6
Q

bence jones proteins

A

myeloma

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7
Q

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

A

roulaeux formation (RBCs staked like a set of coins)

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8
Q

differentials of plasma cell dysplasia (4)

A

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

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9
Q

investigations for plasma cell dysplasia

A

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

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10
Q

what does electrophoresis tell you in plasma cell dysplasia

A

if paraproteins are present

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11
Q

what does serum immunofixation tell you in plasma cell dysplasia

A

classify type of abnormal protein (which Ig)

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12
Q

what is myeloma

A

neoplastic disease of plasma cells

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13
Q

what happens to plasma cells in myeloma

A

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

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14
Q

type of Ig most common in myeloma

A

IgG (can get any)

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15
Q

why does myeloma present with bone pain

A

increased oestoClasts = Chew up bone

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16
Q

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

A

hypercalcaemia

17
Q

presentation of myeloma (5)

A 
bone 
kidney stones 
psychiatric moans 
abdo groans 
thirst/dehydration
18
Q

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

A

myeloma

19
Q

investigations for myeloma

A

electrophoresis
ESR
skeletal survey - to see which bones affected

20
Q

ESR in myeloma

A

raised

21
Q

chronic management of myeloma

A

chemo + steroids/bisphosphonates/thalidomide

22
Q

symptomatic management of myeloma

A

analgesia (NOT NSAIDs bc renal impairment) for pain

bisphosphonates - for bone pain and hypercalcaemia

23
Q

prognosis of myeloma

is it curable

A

not curable

aim for remission, will probs relapse

24
Q

most common cause of paraproteinemia (presence of paraproteins)

A

monoclonal gammopathy of undetermined significance (MGUS)

25
Q

what is monoclonal gammopathy of undetermined significance (MGUS)

A

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

26
Q

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

A

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

27
Q

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

A

v v rarely

28
Q

what happens to the paraproteins in AL amyloidosis

A

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

29
Q

investigations for AL amyloidosis

A

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

30
Q

congo red stain - ‘apple green bifringence’

A

amyloidosis

31
Q

management of AL amyloidosis

how does it work

A

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

32
Q

what type of cancer is waldenstroms macroglobulinaemia

A

non hodgkins lymphoma

33
Q

what type of Ig paraprotein is in waldenstroms

A

IgM

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

34
Q

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

A

IgM is a tetramer = big protein = MACROglobulinaemia

cause hyperviscosity bc so big!

35
Q

presentation of hyperviscosity in waldenstroms macroglobulinaemia

A

bleeding
fatigue
coma
confusion

36
Q

presentation of waldenstroms macroglobulinaemia

A

like lymphoma! - night sweats, weight loss, fatigue

37
Q

investigations for waldenstorms macroglobulinaemia

A

increased viscosity

protein electrophoresis - to see IgM paraproteins

38
Q

management of waldenstroms macroglobulinaemia (3)

A

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

J Haem (12 decks)

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