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Haematology > Myeloma and plasma cell dyscrasias > Flashcards

Myeloma and plasma cell dyscrasias Flashcards

1
Q

Where do B cells come from

A

pluripotent HSC in bone marrow

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2
Q

are B cells part of the innate or adaptive immune system

A

adaptive (slower, more specific response)

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3
Q

what are immunoglobulins

A

antibodies produced by B cells and plasma cells

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4
Q

what are immunoglobulins made of

A

2 heavy chains: IgG/A/M/E/D

2 light chains: k/lambda

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5
Q

each antibody can recognise multiple antigens, true or false

A

false

each antibody recognises a specific antigen

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6
Q

which Ig antibodies are monomeric

A

IgG, IgD, IgE

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7
Q

which Ig antibody is dimeric

A

IgA

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8
Q

which Ig antibody is pentameric

A

IgM

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9
Q

what allows for variability in Ig

A

V-D-J region recombination

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10
Q

Where do B cells in the periphery travel to

A

follicle germinal centres in lymph nodes to identify antigens

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11
Q

what can happen to a B cell after it finishes in the lymph node

A

may return to the bone marrow as a plasma cell or circulate as a memory B cell

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12
Q

what are plasma cells

A

factory cells that pump out antibody

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13
Q

what are histological features of plasma cells

A

eccentric clock face nucleus with H&E staining
open chromatin
plentiful blue cytoplasm
pale perinuclear area

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14
Q

define polyclonal

A

produced by many different plasma cell clones

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15
Q

what can polyclonal proliferation be indicative of

A

infection
autoimmune condition
malignancy - as a reaction from the host to a malignant clone
liver disease

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16
Q

define monoclonal

A

all derived from clonal expansion of a single B cell

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17
Q

characteristics of monoclonal antibodies

A

identical structure and specificity

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18
Q

what are monoclonal antibodies also known as

A

paraprotein

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19
Q

what are paraproteins a marker of

A

underlying clonal B cell disorder

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20
Q

what investigation is used to detect immunoglobulins

A

serum electrophoresis

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21
Q

in serum electrophoresis, what causes proteins to move the way they do

A

their shape and charge

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22
Q

what investigation is used to classify abnormal protein bands found in electrophoresis

A

serum immunofixation

23
Q

What are Bence Jones Proteins BJPs

A

free light chains detected by urine electrophoresis

24
Q

are light chains normally secreted into plasma

A

yes, in very small amounts 0.5g/l

25
Q

what can cause raised free light chains

A

polyclonal (infection) or monoclonal (multiple myeloma) increase in plasma cells

26
Q

causes of paraproteinaemia

A 
myeloma 
lymphoma 
MGUS
amyloidosis
plasmacytoma 
CLL
27
Q

MGUS is benign/malignant

A

benign/premalignant

28
Q

asymptomatic myeloma is benign/malignant

A

malignant - but no organ damage

29
Q

what are the direct tumour effects of myeloma on the body

A

bone lesions
bone pain
hypercalcaemia
marrow failure

30
Q

what are the paraprotein mediated effects of myeloma on the body

A

renal failure
immunosuppression
hyperviscosity
amyloid

31
Q

how can multiple myeloma be classified

A

according to Ig Ab produced

32
Q

why do you get lytic bone lesions in myeloma

A

malignant myeloma cells suppress osteoblasts and activate osteoclasts (also the reason for hypercalcaemia)

33
Q

list consequences of hypercalcaemia

A 
kidney stones 
psychiatric moans
abdominal groans 
painful bones 
thirst and dehydration 
renal impairment
34
Q

how can multiple myeloma lead to renal failure by cast nephropathy

A

free light chains can filter through glomerulus
proximal convoluted tubule cannot deal with light chain overload so they pass to ascending limb of loop of Henle
ascending limb produces Tam-Horsfall proteins which bind to light chains and make them insoluble forming casts
this blocks the nephron

35
Q

what is the prompt management for dealing with cast nephropathy

A

rehydration
stop nephrotoxic drugs
start chemo/steroids to reduce light chain production

36
Q

what is the median age for multiple myeloma

A

65 years old

37
Q

what is the mainstay of treatment for multiple myeloma

A

combination chemotherapy

38
Q

what can be used to monitor response to treatment in multiple myeloma

A

paraprotein levels

39
Q

what is AHSCT (autologous haemopoeitic stem cell transplant)

A

a method of administering very high dose chemotherapy relatively safely

40
Q

many patients dont tend to relapse in multiple myeloma, true or false

A

false

relapse in inevitable

41
Q

how can you control symptoms in multiple myeloma

A

opiate analgesia (not NSAIDs)
local radiotherapy
bisphsphonates
vertebroplasty

42
Q

what does MGUS stand for

A

monoclonal gammopathy of undetermined significance

43
Q

how can you define MGUS

A

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

44
Q

what is AL amyloidosis

A

rare disorder

slowly progressive multisystem disease

45
Q

what is the pathophysiology of AL amyloidosis

A

mutation in light chain causes abnormal structure which precipitates in tissues as insoluble pleated B sheets

46
Q

what organ damage can occur in AL amyloidosis

A 
kidneys - nephrotic syndrome 
heart - cardiomyopathy 
liver - hepatomegaly, deranged LFTs
neuropathy - ANS, PNS
GI tract - malabsorption
47
Q

how can you diagnose AL amyloidosis

A

organ biopsy with congo red stain
(positive result - apple green birefringence under polarised light)
or less invasive rectal/fat biopsy

48
Q

what is a SAP scan for AL amyloidosis

A

serum amyloid P scintigraphy scan can localise amyloid deposits rapidly and specifically

49
Q

what is Waldenstroms macroglobulinaemia

A

lymphoplasmacytoid neoplasm = clonal disorders of cells which are in between a lymphocyte and a plasma cell

50
Q

what is characteristic of Waldenstroms macroglobulinaemia

A

IgM paraprotein

51
Q

what can IgM paraprotein cause in Waldenstroms macroglobulinaemia

A

hyperviscosity

neuropathy

52
Q

what is the treatment of Waldenstroms macroglobulinaemia

A

chemo

plasmapharesis

53
Q

what is plasmapharesis

A

removal of paraprotein from your circulation and replacing with donor plasma

Haematology (26 decks)

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