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Year 5 path SW > Haematology 12: Myeloma And Amyloid, Monocloncal Gammopathy > Flashcards

Haematology 12: Myeloma And Amyloid, Monocloncal Gammopathy Flashcards

(42 cards)

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1
Q

What is monoclonal gammopathy of uncertain significance (MGUS) ?

A
  • IgG/IgA < 30G/L
  • <10% clonal plasma cells
  • No symptoms (CRAB)
  • Genetic errors occur in plasma cell due to infection, inflammation or chromosomal instability.
  • This causes monoclonal accumulation of the plasma cells
  • This is considered harmless but pre-malignant
  • If further mutation, can become multiple myeloma
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2
Q

Which mutation can trigger transformation of MGUS to multiple myeloma ?

A

K-RAS

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3
Q

What are the clinical features of Multiple myeloma ?

A

Calcium - elevated
Renal impairment
Anaemia
Bone lesions

Monoclonal paraprotein (gammaglobulins) 
Bence jones protein in urine (immunoglobulin light chains)
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4
Q

What are some of the characteristic features of Multiple myeloma on histology ?

A

Overcrowding of plasma cells in bone marrow

Plasmacytic cells: Nucleus on one side (eccentric nucleus), dense chromatin, low nuclear to cytoplasmic ratio

Plasmablastic cells: prominent nucleoli, reticular chromatin, less cytoplasm than mature cells

Red cell rouleaux

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5
Q

List 3 characteristic finding of multiple myeloma on Immunohistochemistry ?

A

CD138
CD38
light chain restricted Ig

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6
Q

What is fanconi syndrome ?

A

Renal tubule acidosis with light chain Chrystal depositions

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7
Q

What are the 4 main types of treatment for multiple myeloma ?

A

Cytostatic drug - Melphalan
Steroids
Immuno modulators- Thalidomide
Proteasome inhibitors

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8
Q

List the 3 features required to diagnose Multiple myeloma ?

A
  • Clonal plasma cells on bone marrow biopsy
  • Paraprotein in blood or urine
  • Evidence of end-organ damage - CRAB
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9
Q

List the 3 features that are required to diagnose Smouldering myeloma ?

A
  • Serum paraprotein > 30 g/L
  • Clonal plasma cell > 10 % on bone marrow biopsy
  • No evidence of end organ damage (CRAB)

(This is basically asymptomatic MM)

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10
Q

List 3 features required to diagnose MGUS ?

A
  • Serum paraprotein < 30g/l
  • Clonal plasma cells < 10 % on bone marrow biopsy
  • No signs of end organ damage (CRAB)
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11
Q

List 3 features of waldenstrom macroglobinaemia ?

A
  • Low grade NHL (lymphoplasmacytoid)
  • Monoclonal IgM
  • Hyperviscosity symptoms (visual problems, CCF, weakness, confusion)
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12
Q

list some key features of myeloma plasma cells

A

infiltrate bone marrow
can form expansile or soft tissue tumours - plasmacytomas
produce a serym monoclonal IgG or IgA - paraprotein or M spike
produce excess of monoclonal kappa or lambda free light chains
BJ protein in urine - urine monoclonal free light chains

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13
Q

define multiple myeloma

A

cancer of transformed plasma cells, terminally differentiated B cells, that secrete immunoglobulins and are effector cells of the specific humoural response

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14
Q

describe B cell development

A

common lymphoid progenitor … mature B cell (bone marrow)

mature B cell… short lived plasma cell (marginal zone)

mature B cell … memory cell/ long lived plasma cell (germinal centre)

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15
Q

RF for myeloma

A

obesity
age
genetics

always preceded by a premalignany condition = MGUS

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16
Q

what is MGUS

A 
most common (known) premalignant condition 
incidence increases with age 
average risk of progression 1% annually 
IgG or IgA MGUS --> myeloma
IgM --> lymphoma
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17
Q

diagnostic criteria for MGUS

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A

serum M (monoclonal) protein <30g/l
bone marrow clonal plasma cells <10%
no lytic bone lesions
no myeloma-related organ or tissue impairment
no evidence of other B-cell proliferative disorder

18
Q

RF for MGUS progression

Study These Flashcards
A

non-IgM spike
M spike > 15g/L
abnormal serum free light chain (FLC) ratio

19
Q

what is smouldering myeloma

Study These Flashcards
A

serum monoclonal protein (IgG or IgA) >/= 30g/L or urinary monoclonal protein >/=500mg per 24 hrs and/or clonal bone marrow plasma cells 10-60%

abesence of myeloma deficing events or amyloidosis

20
Q

describe the clinical spectrum of myeloma and related plasma cell disorders

Study These Flashcards
A 
MGUS
smouldering myeloma
symptomatic myeloma
relapsing-remitting 
refractory 
plasma cell leukaemia
21
Q

describe the pathogenesis of MM

Study These Flashcards
A

primary events - hyperdiploidy, IGH rearrangements

secondary events -
KRAS, NRAS…

22
Q

how do myeloma cells interact with the bone marrow microenvironment

Study These Flashcards
A

bone destruction
angiogenesis
anaemia
immunosuppression and infection

23
Q

diagnostic criteria for MM

Study These Flashcards
A

C - hypercalcaemia - Ca >2.75 mmol/L

R - renal disease - creatinine > 177 umol/L or eGFR <40 ml/min

A - anaemia - Hb <100g/L or drop by 20g/L

B - bone disease - two or more bone lytic lesionso

24
Q

presentation of bone disease in myeloma patients

Study These Flashcards
A 
proximal skeleton 
back, chest wall, pelvic pain 
ostrolytic lesions, never osteoblastic 
osteopenia
pathological fractures
hypercalcaemia
25
what bone imaging can we do in myeloma
plain XR films whole body CT CT/FDG-TER whole body diffusion weighted MRI
26
what bone disease emergencies to we get in MM
cord compression - diagnose and treat within 24 hrs, MRI, dexamethasone, radiotherapty, stabilise unstable spine hypercalcaemia - dowsiness, constipation, fatigue, muscle weakness, AKI Give fluids, steroids, zolendronic acid
27
define myeloma kidney disease
serum creatinine >177 umol/l OR eGFR <40ml/min AKI as a result of myeloma
28
what are the causes of myeloma kidney disease
cast nephropathy cause by high serum free light chain (FLC) levels and BJP hypercalcaemia, loop diuretics, infection, dehydration, nephrotoxics
29
what medication is the cornerstone of myeloma kidney disease treatment
bortezomib
30
why do we get infections in myeloma
``` humoral and cellular immunodeficiency myeloid, T and NK impairment immunoparesis: low serum normal Igs chemo impairs the immune response myeloma immune evasion ```
31
how can we diagnose myeloma
immunoglobulin studies: - serum protein electrophoresis - serum free light chain levels - 24hr BJP BM aspirate and biopsy: - IHC fod CD138 FISH analysis Flow cytometry immunophenotyping: - MRD (detection of minimal residue disease)
32
describe the myeloma plasma cell phenotype
``` CD319 (SLAM7) ++ CD56 ++ cytoplasmic Ig +++ CD38 +++ CD138 +++ BCMA ++ ```
33
how is myeloma staged
ISS | international staging system
34
how can myeloma result in AL amyloidosis
MGUS or myeloma in the background misfolded free light chains aggregate into amyloid fibrils in target organs amyloid potential of light chains is more important than the amount amyloid fibrils stain with congo red lambda light chain involvement in 60%
35
clinical presentation of AL amyloidosis
``` kidney, heart, liver, neuropathy - nephrotic syndrome - proteinuria and peripeheral oedema unexplained HF - raised NT-proBNP sensory neuropathy abnormal LFTs macroglossia ```
36
what is MGRS
monoclonal gamaopathy of renal significance any B cell clonal lymphoproliferation where there are - one or more kidney lesions caused by raised Ig AND - the underlying B cell clone does no cause tumour complications or meet the haem criteria for specific therapy
37
what therapies are used in myeloma treatment
melphalan cyclophosphamide - in combination with steroids dexamethasone + pred - induces apoptosis in myeloma cells
38
which immunomodulatory drugs can be used in myeloma
lenalidomide | promalidomide
39
how can proteasome inhibitors treat myeloma
myeloma cells are protein production factories proteasome is crucial in removing misfolded proteins accumulation of misfolded proteins - ER stress and unfolded protein response - apoptosis eg bortezomib, carflizombib, ixazomib
40
treatment algorithm for transplant eligible MM pt
induction - PI + iMiD + Dex + autologous SC transplant + consolidation x2 + maintenance low dose lenalidomide
41
treatment algorithm for transplant-ineligible patients
``` lenalidomide + dex or bortezomib -cyclophosphamide - dex or daratumumab - bartezomib - cyclophosphamide - pred ```
42
new MM treatments
CAR-T cell therapy, BiTE and immunoconjugates

Year 5 path SW (106 decks)

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