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Haematology > Myeloma & Plasma Dyscrasia > Flashcards

Myeloma & Plasma Dyscrasia Flashcards

1
Q

What are immunoglobulins?

A

Antibodies - proteins produced by B cells and plasma

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2
Q

Describe the structure of immunoglobulins

A

Two heavy and two light chains bound by a disulphide bond. Each have a specific antigen binding site

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3
Q

Describe the different shapes of antibody

A

Monomer - IgD, IgE, IgG
Dimer - IgA
Pentamer - IgM

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4
Q

Where are B cells produced?

A

Bone marrow

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5
Q

How does the variable element of B cells arise?

A

V-D-J recombination

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6
Q

What happens to B cells when they leave the bone marrow?

A

Immature B cells with Ig on surface leave and go onto meet their target antigen - IgM

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7
Q

Where does the B cell encounter the antigen?

A

Follicle germinal centre of lymph node

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8
Q

What happens to the B cell in the lymph node?

A

Identifies the antigen
Improve the fit by mutation or deletion
Class switching

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9
Q

After the lymph node interactions what can happen to the B cell?

A

Return to the marrow as a plasma cell

Remain in circulation as memory B cell

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10
Q

What is the function of plasma cells?

A

Produce antibodies

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11
Q

Describe the appearance of plasma cells

A

Eccentric clock face nucleus on H and E stain
Open chromatin (synthesising mRNA)
Blue cytoplasm with lots of protein
Pale perinuclear area due to golgi apparatus

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12
Q

What do plasma cells look like on bone marrow aspirate?

A

Fried Egg

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13
Q

What is meant by polyclonal?

A

Lots of clones encounters specific antigen and ready to produce antibodies

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14
Q

What does a polyclonal immunoglobulinopathy imply?

A

Reactive aetiology

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15
Q

Name the causes of polyclonal immunoglobulinopathy

A

Infection
Autoimmune
Malignancy
Liver disease

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16
Q

What do monoclonal immunoglobulinopathies produce?

A

Paraprotein

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17
Q

How can monoclonal immunoglobulinopathies be investigated?

A

Serum electrophoresis
Immunofixation
Quantified by graph calculation

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18
Q

Describe serum electrophoresis

A

Sample serum on gel and apply voltage different proteins move different distances
Ig found in gamma region and should be diffuse more distinct band will appear in the presence of paraprotein

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19
Q

What does immunofixation do?

A

Mix with anti-sera that binds to heavy or light chains to classify Ig

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20
Q

What protein can be identify by urine electrophoresis?

A

Bence Jones Protein

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21
Q

What is BJP?

A

Excess light chains leak into the urine and precipitate

22
Q

Name the causes of paraproteinaemia

A 
MGUS 
Myeloma 
Amyloidosis 
Waldenstroms
Leukaemia/NHL
23
Q

What is myeloma?

A

Cancer of plasma cells

24
Q

Describe the progression to myeloma

A

Normal
MGUS
Asymptomatic
Overt myeloma

25
Q

What are the direct tumour effects of myeloma?

A

Bone lesions, increased calcium, bone pain, marrow failure (pancytopenia)

26
Q

How can bone lesions present?

A

Focal erosion of bone - pepper pot skull

Compression fractures in spine

27
Q

What are the paraprotein mediated effects of myeloma?

A

Renal failure
Immune suppression
Hyperviscosity
Amyloid

28
Q

What is the commonest myeloma?

A

IgG

29
Q

How does myeloma cause bone lesions?

A

Malignant cells produces cytokines that activate osteoclasts and suppress osteoblasts
Osteoclasts produce cytokines which stimulate more myeloma cells

30
Q

Describe hypercalcaemia

A 
Stones
Bones 
Groans 
Psych moans
Thirst 
Dehydration 
Renal impairment
31
Q

What is the effect of myeloma on the kidney?

A 
Tubular damage by light chains 
Precipitate causing casts 
Risk of sepsis 
NSAIDs --> AKI 
Hyperuricaemia due to high cell turnover 
Amyloid
32
Q

What are casts?

A

Solid aggregates - light chains bind to Tamm horsfall protein causing obstructive uropathy

33
Q

How is obstructive uropathy due to casts treated?

A

Hydration, stop nephrotoxic drugs, switch off light chain production with steroids/chemo

34
Q

What is the median age of myeloma diagnosis?

A

65 years old

35
Q

How is myeloma treated?

A 
Combination chemotherapy 
- corticosteroids 
- alkylating agents 
- novel agents 
- monoclonal antibodies 
Autologous stem cell transplant
36
Q

How do you monitor response to myeloma treatment?

A

Measure paraprotein

37
Q

Describe the symptom control of myeloma

A

Opiate analgesia
Local radiotherapy
Bisphosphonates
Vertebroplasty

38
Q

What does MGUS stand for?

A

Monoclonal gammopathy of undetermined significance

39
Q

How is MGUS different to myeloma?

A

Paraprotein <30g/l
Fewer faulty plasma cells <10% of bone marrow plasma
No end organ damage
Normal calcium, renal function, Hb, no lytic lesions, no increased infections

40
Q

Describe the pathology of AL amyloidosis

A

Small plasma cell clone - faulty light chain has the potential to misfiled and aggregate to be insoluble - beta pleated sheets become amyloid fibril

41
Q

What happens as fibrils accumulate>

A

Build up in tissues and cause organ damage

42
Q

How is AL amyloidosis treated?

A

Chemotherapy

43
Q

For each organ describe the damage AL amyloid causes

A 
Kidney - nephrotic syndrome 
Heart - cardiomyopathy 
Liver - organomegaly, LFT abnormality 
Neuropathy - autonomic/peripheral 
GI - malabsorption
44
Q

How is AL amyloid diagnosed?

A

Biopsy - bone marrow, fat, renal - congo red stain which apple green birefringence on microscopy

45
Q

Name the indirect scans for AL amyloid

A

SAP scan
Echo/MRI
Nephrotic range proteinuria

46
Q

What is Waldenstrom’s Macroglobulinaemia?

A

Lymphoplasmactyoid neoplasm - clonal disorder of cells between lymphocyte and plasma

47
Q

What are the tumour effects of Waldenstrom’s Macroglobulinaemia?

A

Lymphadenopathy
Splenomegaly
Marrow failure

48
Q

What paraprotein is in Waldenstrom’s Macroglobulinaemia?

A

IgM

49
Q

Describe the paraprotein effects of Waldenstrom’s Macroglobulinaemia

A

Hyperviscosity

Neuropathy

50
Q

How does hyperviscosity present?

A

High levels of IgM

  • fatigue
  • visual disturbance
  • confusion
  • bleeding
  • cardiac failure
  • coma
  • B symptoms
51
Q

How is Waldenstrom’s Macroglobulinaemia treated?

A

Chemotherapy

Plasmaphoresis - removes paraprotein and replaces with donor FFP

Haematology (18 decks)

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