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Haematology > Plasma Cell Dyscrasias > Flashcards

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

A

0.5g/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
Q

how is myeloma classified

A

by the type of antibody produced

26
Q

what is the most common type of antibody produced in myeloma

A

IgG (60%), IgA (21%), Bence Jones myeloma-free light chain (15%)

27
Q

what substance does myeloma release that can cause symptoms

A

myeloma forms lytic bone lesions which releases calcium and this can cause hypercalcaemia

28
Q

what are the symptoms of hypercalcaemia

A

stones, bones, abdo groans, psychiatric moans, thirst, dehydration, renal impairment

29
Q

what percentage of patients with myeloma have renal impairment at diagnosis

A

30%

30
Q

which part of the kidney can resorb and catabolise the light chains

A

proximal tubule

31
Q

what happens if there is lots of free chains

A

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
Q

how can cast nephropathy be treated

A

steroids

33
Q

how is myeloma treated

A

corticosteroids, dexamethasone or prednisolone

alkylating agents like thalidomide, vortezomib and lenalidomide

34
Q

how do you monitor response to myeloma treatment

A

paraprotein levels

35
Q

how do you treat the symptoms of myeloma

A

opiate analgesia
local radiotherapy
bisphosphonates
vertebroplasty-inject sterile cement into fractured bone to stabilise

36
Q

what does MGUS stand for

A

monoclonal gammopathy of uncertain significance-MGUS

37
Q

whats the definition of MGUS

A

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
Q

what is the risk of progression to myeloma with MGUS

A

1%

Haematology (14 decks)

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