Plasma Cell Dyscrasias Flashcards

1
Q

what are the role of B cells

A

antibody production and 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

2 heavy and 2 light chains

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4
Q

what does an antibody recognise

A

a specific antigen

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5
Q

the Ig variable element comes form where

A

V-D-J region recombination early in development

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6
Q

what do B cells look like when they leave the marrow

A

immature B cells with immunoglobulin on their surface exit bone marrow

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7
Q

pre B cells produce what type of im

A

ImM and ImD

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8
Q

where do B cells travel to in the periphery

A

follicle germinal centre of the lymph node

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9
Q

what happens to B cells after they’ve traveled

A

return to the marrow as plasma cells or circulate as memory cells

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10
Q

appearance of a plasma cell

A

eccentric ‘clock face nucleus on H and E
open chromatin-synthesing mRNA
plentiful blue cytoplasma-laden with protein
pale perinuclear area

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11
Q

polyclonal immunoglobulins are produced in response to what

A

infection
autoimmune
malignancy
liver disease

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12
Q

what does monoclonal immunoglobulin=

A

paraprotein

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13
Q

what does paraptorein indicate

A

marker of an underlying clonal B cell disorder

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14
Q

how are immunoglobulins detected

A

serum electrophoresis

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15
Q

what does electrophoresis detect

A

abnormal protein bands

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16
Q

what is Bence Jones Protein

A

immunoglobulin light chains

17
Q

how are bence jones protiens detected

A

urine electrophoresis

18
Q

free light chain production by normal plasma cells is how much a day

19
Q

what happens to excess light chains

A

they leak into the urine as BJP

20
Q

what are the 2 types of light chains

A

kappa and lambda

21
Q

what are the main causes of paraproteinaemia

A
MGUS=56%
myeloma=18%
amyloidosis 10%
lymphoma=5%
asymptomatic myeloma=4%
chronic lymphocytic leukaemia=2%
waldenstrom's macroglobulinaemia=2%
22
Q

what are the 2 types of effects myeloma has

A

direct tumour cell effects

paraprotein mediated effects

23
Q

what are the direct tumour cell effects of myeloma

A

bone lesions, increased calcium, bone pain, replace normal bone marrow-marrow failure

24
Q

what are some paraprotein mediated effects

A

renal failure
immune suppression
hyperviscosity
amyloid

25
how is myeloma classified
by the type of antibody produced
26
what is the most common type of antibody produced in myeloma
IgG (60%), IgA (21%), Bence Jones myeloma-free light chain (15%)
27
what substance does myeloma release that can cause symptoms
myeloma forms lytic bone lesions which releases calcium and this can cause hypercalcaemia
28
what are the symptoms of hypercalcaemia
stones, bones, abdo groans, psychiatric moans, thirst, dehydration, renal impairment
29
what percentage of patients with myeloma have renal impairment at diagnosis
30%
30
which part of the kidney can resorb and catabolise the light chains
proximal tubule
31
what happens if there is lots of free chains
in the thick ascending limb in loop of henle, Tamm-Horsfall protein is produced and this can combine with free light chains to produce insoluble casts which block the nephron
32
how can cast nephropathy be treated
steroids
33
how is myeloma treated
corticosteroids, dexamethasone or prednisolone | alkylating agents like thalidomide, vortezomib and lenalidomide
34
how do you monitor response to myeloma treatment
paraprotein levels
35
how do you treat the symptoms of myeloma
opiate analgesia local radiotherapy bisphosphonates vertebroplasty-inject sterile cement into fractured bone to stabilise
36
what does MGUS stand for
monoclonal gammopathy of uncertain significance-MGUS
37
whats the definition of MGUS
PARAPROTEIN <30g/l bone marrow plasma cells <10% no evidence of myeloma end organ damage-normal ca, renal function, normal hb, no lytic lesions, no increase in infections
38
what is the risk of progression to myeloma with MGUS
1%