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3 HAEM > myeloma and other dyscrasia > Flashcards

myeloma and other dyscrasia Flashcards

1
Q

what is the function of B cells

A

antibody production

acting as antigen presenting cells

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2
Q

what are immunoglobulins

A

antibodies produced by B-cells and plasma cells

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3
Q

what are immunoglobulins made up of

A

proteins
2 heavy chains (u, alpha, delta, gamma, epsilon)
2 lights chains (K or lambda)

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4
Q

which immunoglobulins are monomeric

A

IgD
IgE
IgG

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5
Q

which immunoglobulins are dimeric

A

IgA

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6
Q

which immunoglobulins are pentameric

A

IgM

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7
Q

where does initial production and development of B cells occur

A

in the bone marrow

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8
Q

at what stage of development are B cells when they leave the marrow

A

immature B cells with Ig on their surface

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9
Q

what do B cells do in the periphery

A

travel to the follicle germinal centre of the lymph node

identify antigen and improve fit by somatic mutation or deletion

may return to marrow as plasma cell or circulate as memory cell

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10
Q

what do plasma cells do

A

produce lots of antibodies

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11
Q

what are features of plasma cells

A

eccentric ‘clock face’ nucleus
open chromatin
plentiful blue cytoplasm
pale perinuclear area

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12
Q

what is the difference between polyclonal and monoclonal

A

polyclonal cells/antibodies come from many different parent cells

monoclonal cells/antibodies all come from a single precursor

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13
Q

what might cause a polyclonal increase in immunoglobulins

A

infection
autoimmune conditions
malignancy (reaction of the host to malignant clone)
liver disease

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14
Q

what is another word for monoclonal immunoglobulin

A

paraprotein

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15
Q

what are monoclonal immunoglobulins a marker of

A

underlying clonal B-cell disorder

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16
Q

how are immunoglobulins detected/analysed

A

serum electrophoresis

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17
Q

what is the purpose of serum immunofixation

A

to classify the abnormal protein band

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18
Q

what are bence jones proteins

A

immunoglobulin light chains present in urine

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19
Q

how are bence jones proteins detected

A

urine electrophoresis

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20
Q

are free light chains pathological?

A

free light chain production of 0.5 g/day is normal

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21
Q

why is there physiological free light chain production

A

plasma cells produce more light chains than heavy chains so free light chains get secreted into the plasma

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22
Q

are free light chains monomeric or dimeric

A

kappa light chains are monomeric

lambda light chains are dimeric

23
Q

what are causes of paraproteinaemia (monoclonal antibodies)

A 
MGUS (asymptomatic)
myeloma 
amyloidosis
lymphoma
chronic lymphocytic leukaemia
24
Q

what is myeloma

A

a plasma cell malignancy

25
Q

what are direct tumour cell effects of myeloma

A

bone lesions
increased calcium
bone pain
marrow failure due to replacement of normal marrow

26
Q

what are paraprotein mediated effects of myeloma

A

renal failure
immunosuppresion
hyperviscosity
amyloid

27
Q

what is the most common type of myeloma

A

IgG

followed by IgA, then bence jones myeloma

28
Q

how does myeloma cause lytic bone disease

A

myeloma cells secrete IL-6 which causes osteoblast suppression and osteoclast activation
(also releases calcium)

29
Q

what are symptoms of hypercalcaemia

A

stone, bones, abdominal groans and psychiatric moans

thirst, dehydration and renal impairment

30
Q

how does myeloma cause renal failure

A

tubular cell damage by light chains
light chain deposition causes cast nephropathy
sepsis, hypercalcaemia, dehydration, drugs

31
Q

in which part of the nephron does cast nephropathy occur

A

thick ascending limb of loop of Henle

32
Q

how does cast nephropathy occur

A

light chains react with proteins produced in the thick limb of loop of henle to produce insoluble casts that block the nephron

33
Q

how can cast nephropathy potentially be reversed

A

hydration
stop nephrotoxic drugs
steroids/chemo to prevent light chain production

34
Q

how is myeloma managed

A

combination chemotherapy is the mainstay

steroids, alkylating agents, new agents

35
Q

examples of alkylating agents

A

cyclophosphamide

melphalan

36
Q

examples of new chemotherapy agents used in myeloma treatment

A

thalidomide
bortezomib
lenalidomide

37
Q

which myeloma treatment is suitable for fit patients

A

high dose chemo and autologous stem cell transplant

38
Q

what is used to monitor response to myeloma treatment

A

paraprotein level

39
Q

what are the steps involved in autologous haematopoietic stem cell transfer

A

administer pre-treatment to release blood stem cells from bone marrow into blood stream

collect stem cells and freeze until needed

administer chemo to remove/partially remove immune system

return stem cells by infusion into vein

provide supportive treatment until immune system rebuilds

40
Q

how are symptoms controlled in myeloma

A

opiate analgesia
local radiotherapy (pain relief/spinal cord compression)
bisphosphonates (correct hypercalcaemia and bone pain)
vertebroplasty (sterile cement to stabilised fractured bone)

41
Q

what is MGUS

A

monoclonal gammopathy of undetermined significance

42
Q

how is MGUS defined

A

paraprotein <30 g/l
bone marrow plasma cells <10%
no evidence of myeloma end organ damage

43
Q

what causes AL amyloidosis

A

mutation in light chain causing altered structure

results in precipitates forming in tissues as an insoluble beta pleated sheet

44
Q

how is AL amyloidsis treated

A

chemo to switch of light chain supply

45
Q

which organs can be damaged in AL amyloid

A

kidney: nephrotic syndrome
heart: cardiomyopathy
liver: organomegaly and deranged LFTs
neuropathy: autonomic/peripheral
GI tract: malabsorption

46
Q

how is amyloid diagnosed

A

congo red stain on organ biopsy (rectal or fat biopsy is less invasive)

evidence of deposition in other organs
SAP scan
echo/cardiac MRI
nephrotic range proteinuria

47
Q

what does a SAP scan do

A

radio-labelled serum amyloid P localises rapidly and specifically to amyloid deposits in proportion to the quantity of amyloid present

48
Q

what are SAP scan used for

A

monitor disease burden and treatment response

49
Q

what is the buzzword for amyloid in Congo red staining

A

‘apple-green’ birefringence under polarised light

50
Q

what cells are affected in Waldenstrom’s macroglobulinaemia

A

clonal disorder of cells intermediate between a lymphocyte and a plasma cell

51
Q

which Ig is produced in Waldenstrom’s macroglobulinaemia

A

IgM

pentameric = large = macroglobulin

52
Q

what are clinical features of waldenstrom’s

A

hyper viscosity syndrome: fatigue, visual disturbance, confusion, coma, bleeding, cardiac failure
night sweats
weight loss

53
Q

how is waldenstrom’s treated

A 
chemo
plasmapheresis (removes paraprotein from the circulation)

3 HAEM (20 decks)

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