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Haematology > Myeloma and Plasma Cell Dyscrasias > Flashcards

Myeloma and Plasma Cell Dyscrasias Flashcards

1
Q

what is the role of the B cells

A

part of adaptive immune system:

  • antibody production
  • antigen presenting cells
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2
Q

what are immunoglobulins

A

antibodies produced by B cells and plasma cells
are proteins made up of 2 heavy and 2 light chains
each antibody recognises a specific antigen

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3
Q

what is the variable region of an immunoglobulin

A

the antigen binding site

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4
Q

how do B cells develop

A

initial production and development in the bone marrow (under influence of microenvironment)
Ig variable element generated (vjd segment)
self reactive cells removed
immature B cells with immunoglobulin (Ig) on their surface exit bone marrow

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5
Q

how do b cells circulate

A

travel to the follicle germinal centre of the lymph node
identify the antigen and improve the fit by somatic mutation or be deleted
may return to marrow as plasma cell or circulate as a memory cell

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6
Q

what is a plasma cell

A

short lived B cell that produces antibodies

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7
Q

what is the structure of a plasma cell

A 
clock face nucleus 
open chromatin 
blue cytoplasm 
pale 
pale perinuclear area (golgi apparatus)
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8
Q

what causes a polyclonal increase in immunoglobulins

A

(produced by many different cell clones)
reactive to:
-infection
-autoimmune
-malignancy (reaction of host to malignant clone)
-liver disease

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9
Q

what is a monoclonal rise in immunoglobulin

A

all derived from clonal expansion of a single B cell

identical antibody structure and specificity

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10
Q

what is paraprotein

A

monoclonal immunoglobulin

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11
Q

what is paraprotein a marker of

A

underlying clonal B cell disorder

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12
Q

how can you detect immunoglobulins

A

serum electrophoresis (detects abnormal protein bands)

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13
Q

what does serum immunofixation do

A

classifies the abnormal protein bands

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14
Q

what is bence jones protein

A

immunoglobulin light chains, when excess leak into urine

detected by urine electrophoresis

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15
Q

what can cause an excess (>0.5g/day) of free light chain by normal plasma cells

A

anything that causes an increase in immunoglobulin synthesis (polyclonal: e.g. infection or monoclonal: e.g. multiple myeloma)

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16
Q

what causes paraproteinaemia

A 
monoclonal gammopathy of unknown significance 
myeloma 
amyloidosis (AL)
lymphoma 
chronic lymphocytic leukaemia
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17
Q

what is myeloma

A

plasma cell malignancy

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18
Q

what is MGUS

A

premalignant stage before asymptomatic myeloma

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19
Q

what is asymptomatic myeloma

A

when there is cancer but no organ damage

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20
Q

how does myeloma affect the body

A

clonal plasma cells:

  • direct tumour cell effects
  • paraprotein mediated effects
21
Q

what are the direct tumour cell effects of myeloma

A

bone lesions
increased calcium
bone pain
replace normal bone marrow: marrow failure

22
Q

what are the paraprotein mediated effects of myeloma

A

renal failure
immune suppression
hyperviscosity
amyloid

23
Q

how is myeloma classified

A

by the type of antibody produced:

  • IgG (59%)
  • IgA (21%)
  • bence jones myeloma (15%)
24
Q

what does lytic bone disease look like

A

multiple punched out lytic lesions (myeloma)

wedge compression #s

25
Q

what causes myeloma lytic bone disease

A

osteoblasts suppressed

osteoclasts activated

26
Q

what are the features of hypercalcaemia

A 
stones 
bones 
abdominal groans 
psychiatric moans 
thirst 
dehydration 
renal impairment
27
Q

how does myeloma affect the kidney

A 
light chains cause tubular damage 
light chain deposition: cast nephropathy (in thick ascending limb)
sepsis 
hypercalcaemia and dehydration 
hyperuricaemia
amyloid
28
Q

what is the treatment for cast nephropathy

A

hydration
stop nephrotoxic drugs (NSAIDs)
switch off light chain production with steroids/ chemo

29
Q

what is the treatment for myeloma

A

combination of chemo
corticosteroids
alkylating agents (e.g. cyclophosphamide)
novel agents (thalidomide)

HIGH DOSE CHEMO/ AUTOLOGOUS STEM CELL TRANSPLANT in fit patients

30
Q

what is the median age of myeloma diagnosis

A

65

31
Q

how do you monitor myelomas response to treatment

A

paraprotein levels

32
Q

what is autologous haematopoietic stem cell transplant

A

administer re treatment to release stem cells from marrow into blood stream
collect the stem cells and freeze until needed
administer chemo
return thawed stem cells via transfusion
(way of delivering high dose chemo safely)

33
Q

what is the prognosis for myeloma

A

5-10 years for younger patients

relapse is inevitable

34
Q

what can be done for symptoms control in myeloma

A 
opiate analgesia (avoid NSAIDs)
local radiotherapy (pain relief/ spinal cord compression) 
bisphosphonates (corrects hypercalcaemia and bone pain) 
vertebroplasty (inject sterile cement into fractured bone to stabilise)
35
Q

what is MGUS

A 
when paraprotein <30 
bone marrow plasma cells <10%
no evidence of myeloma end organ damage: 
-normal calcium 
-normal renal function 
-normal Hb
-no lytic lesions 
-no increase in infection
36
Q

how many MGUS progress to myeloma

A

1% per year

37
Q

what is AL amyloidosis

A

amyloid light chain amyloidosis
rare disorder where a small plasma cell clone has a mutation in the light chain= precipitates in tissues as an insoluble beta pleated sheet

38
Q

how does AL amyloidosis present

A

organ damage

slowly progressive

39
Q

what is SAA amyloidosis

A

caused by chronic inflammation

40
Q

what is the treatment for AL amyloidosis

A

chemo (to switch of light chain supply)

41
Q

what organs are damaged in AL amyloidosis

A 
kidney (nephrotic syndrome) 
heart (cardiomyopathy) 
liver (organomegaly, deranged LFTs)
neuropathy (autonomic, peripheral) 
GI tract (malabsorption)
42
Q

how is amyloid diagnosed and staged

A

organ biopsy confirming AL amyloid deposition - congo red stain, rectal/ fat biopsy done if high clinical suspicion

to stage: look for evidence of deposition in other organs:

  • SAP scan
  • echo/ cardiac MRI
  • nephrotic range proteinuria
43
Q

what is used to monitor AL amyloid response to Tx

A

SAP scan- monitors disease burden and response

44
Q

what is seen on congo red staining in AL amyloid

A

apple green birefringence under polarised light

45
Q

what is waldenstroms macroglobulinaemia (IgM paraprotein)

A 
lymphoplasmacytoid neoplasm (clonal disorder of cell inbetween a lymphocyte and plasma cell)
IgM paraprotein
46
Q

what are the effects of IgM paraprotein

A

tumour effects:

  • lymphenopathy
  • splenomegaly
  • marrow failure

paraprotein effects:

  • hyperviscosity
  • neuropathy
47
Q

what shape is the IgM antibody

A

pentameric

48
Q

what are the clinical features of waldenstroms

A

hyperviscosity syndrome:

  • fatigue visual disturbance, confusion, coma
  • bleeding
  • cardiac failure

b symptoms: night sweats, weight loss

49
Q

what is the treatment for waldenstroms

A 
chemo 
plasmapheresis (remove patient plasma (rich in IgM paraprotein) replace with donor plasma)

Haematology (27 decks)

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