Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Haematology > Myeloma and Other Plasma Cell Dyscrasias > Flashcards

Myeloma and Other Plasma Cell Dyscrasias Flashcards

1
Q

What are B cells derived from?

A

Pluripotent haematopoietic stem cells in bone marrow

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is the role of B cells?

A

Part of adaptive immune system

Dual roles: antibody production, acting as APCs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are Immunoglobulins?

A

Antibodies produced by B or plasma cells
Proteins made up of 2 heavy ((μ, α, δ, γ, ε) and 2 light chains (κ or λ)
Each Ab recognises a specific Ag

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is the structure of IgD, E and G?

A

Monomer

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is the structure of IgA?

A

Dimer

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is the structure of IgM?

A

Pentamer

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is the Ig variable element of B-cells generated from?

A

V-D-J region recombination early in development

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Describe B cell development

A

Initial production/development in marrow
Under control/influence of microenvironment
Ig variable element generated
Self-reactive cells removed
Immature B cells with Ig on their surface exit bone marrow ready to meet target

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

From IgM Pro B cells, what is then produced?

A 
IgM and IgD Pre B cells in marrow, then:
IgM B cells, which produce
IgM plasma cells
IgA
IgE
IgG, which produce plasma cells
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Describe B cells in the periphery

A

Travel to the follicle germinal centre of the LN
Identify the antigen and improve the fit by somatic mutation or be deleted
May return to the marrow as plasma cell or circulate as memory cell

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Describe plasma cells

A 
Factory cells
Pumps out Ab
Eccentric clock face nucleus on H&E
Open chromatin- synthesising mRNA
Plentiful blue cytoplasm- laden with protein
Pale perinuclear area- Golgi apparatus
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What will polyclonal Ig’s be reactive to?

A

Infection
AI
Malignancy-reaction of host to malignant clone
Liver disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is a monoclonal Ig?

A

Paraprotein- marker of underlying clonal B-cell disorder

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

How are Ig’s detected?

A

Serum electrophoresis- separated serum proteins appear as distinct bands or zones

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the most -vely charged molecule found on electrophoresis?

A

Albumin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is contained in the alpha-1 band?

A

Alpha 1 antitrypsin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is contained in the alpha 2 band?

A

Alpha 2 macroglobulin, caeruloplasmin, haptoglobin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is contained in the beta band?

A

Transferrin, low density lipoprotein, C3

19
Q

What is contained in the gamma band?

A

IgG, A, M, D, E

20
Q

What is serum immunofixation used for?

A

To classify the abnormal protein band

21
Q

What are Bence-Jones protein?

A

Ig light chains, detected by urine electrophoresis

22
Q

What amount of free light chain is produced by normal plasma cells each day?

A

0.5g/day

Excess can leak into the urine as BJP

23
Q

What are the causes of paraproteinaemia?

A 
MGUS 56%
Myeloma 18%
Amyloidosis 10%
Lymphoma 5%
Asymptomatic myeloma 4%
Solitary or extramedullary plasmacytoma 3%
CLL 2%
Waldenstroms macroglobulinaemia 2%
24
Q

What are the direct tumour cell effects of clonal plasma cells in myeloma?

A

Bone lesions
Increased calcium
Bone pain
Replacement of normal bone marrow- leading to marrow failure

25
Q

What are the paraprotein mediated effects of clonal plasma cells in myeloma?

A

Renal failure
Immunosuppression
Hyperviscosity
Amyloid

26
Q

How is myeloma classed?

A

Type of Ab produced

27
Q

What are the types of myeloma?

A 
IgG 56%
IgA 21%
BJP myeloma- free light chain 15%
Nonsecretory myeloma 3%
IgD 1%
Biclonal 1%
IgE 0.01%
28
Q

Why does myeloma cause lytic bone disease?

A

Increased levels of IL-6, leading to increased TGFbeta, osteoblast suppression and osteoclast activation
Leads to hypercalcaemia

29
Q

What are the symptoms and signs of hypercalcaemia?

A 
Stones
Bones
Abdominal groans
Psychiatric moans
Thirst
Dehydration
Renal impairment
30
Q

What causes damage to the kidney in myeloma?

A 
Tubular cell damage by light chains  
Light chain deposition; cast nephropathy   
Sepsis
Hypercalcemia and dehydration
Drugs; NSAIDs
Amyloid
Hyperuricaemia
31
Q

How is cast nephropathy managed?

A

Damage may be reversible with prompt treatment

Switch off light chain production with steroids/chemo

32
Q

What is the treatment of myeloma?

A

Corticosteroids; dexamethasone or prednisolone
Alkylating agents eg cyclophosphamide, melphalan
‘Novel agents’ like thalidomide, bortezomib and lenalidomide
Monoclonal antibodies against plasma cells
High dose chemo/autologous stem cell transplant in fit patients

33
Q

How is myeloma response to treatment monitored?

A

Paraprotein level

34
Q

How are myeloma symptoms controlled?

A

Opiate analgesia (avoid NSAIDs)
Local radiotherapy - good for pain relief or spinal cord compression
Bisphosphonates - corrects hypercalcaemia and bone pain
Vertebroplasty – inject sterile cement into fractured bone to stabilise

35
Q

What is the definition of monoclonal gammopathy of uncertain significance (MGUS)?

A 
Paraprotein <30g/l
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 infections
36
Q

What is the risk of progression to myeloma of MGUS?

A

~1%

37
Q

Describe Amyloid light chain (AL) Amyloidosis

A

Rare
Small plasma cell clone
Mutation in light chain, leads to altered structure
Precipitates in tissues as an insoluble beta pleated sheet
Often presents late with organ damage
Accumulation in tissues causes organ damage
Slowly progressive, multisystem
Different protein to SAA amyloidosis/familial amyloidosis
Poor prognosis esp if cardiac amyloid

38
Q

What organ damage can AL amyloidosis cause?

A 
Kidney- Nephrotic syndrome
Heart- Cardiomyopathy
Liver- Organomegaly deranged LFT’s
Neuropathy-Autonomic, Peripheral
GI tract- Malabsorption
39
Q

How is AL Amyloidosis diagnosed and staged?

A

Organ biopsy- congo red stain, rectal or fat biopsy may be done if high clinical suspicion as less invasive
Evidence of deposition in other organs- SAP scan, echo, heavy proteinuria

40
Q

What is Waldenstrom’s Macroglobulinaemia(IgM paraprotein)?

A

Lymphoplasmacytoid neoplasm- clonal disorder of cells intermediate between a lymphocyte and plasma cell, characteristic IgM paraprotein

41
Q

What are the tumour effects of Waldenstrom’s?

A

Lymphadenopathy
Splenomegaly
Marrow failure

42
Q

What are the paraprotein effects of Waldenstrom’s?

A

Hyperviscosity

Neuropathy

43
Q

What are the clinical features of WM?

A

Hyperviscosity syndrome- fatigue, visual disturbance, confusion, coma, bleeding, cardiac failure
B symptoms: night sweats, weight loss

44
Q

How is WM treated?

A 
Chemo
Plasmapheresis (removes paraprotein from circulation)

Haematology (23 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions