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Year 3 - Haematology > Myeloma and Plasma Cell Dyscrasias > Flashcards

Myeloma and Plasma Cell Dyscrasias Flashcards

1
Q

What are immunoglobulins composed of?

A

2 heavy chains and 2 light chains

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2
Q

Which immunoglobulins ae monomers?

A

IgD
IgE
IgG

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3
Q

Which immunoglobulins are dimers?

A

IgA

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4
Q

Which immunoglobulins are pentamers?

A

IgM

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5
Q

Where are B cells produced?

A

bone marrow

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6
Q

What becomes the antigen binding site on the immunoglobulin?

A

Random selection of V D and J genes

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7
Q

Where do B cells travel to get antigens?

A

Follicle germinal centre of the lymph node

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8
Q

What is found in the pale perinuclear area of a plasma cell?

A

Golgi apparatus

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9
Q

Describe the nucleus of a plasma cell

A

Clock-face

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10
Q

Which scenarios would cause a polyclonal increase in immunoglobulins?

A

Infection
Autoimmunity
Malignancy
Liver disease

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11
Q

Give examples of causes of paraproteinaemia?

A 
MGUS
Amyloidosis 
Myeloma
Lymphoma
CLL
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12
Q

How are immunoglobulins detected in the blood?

A

Electrophoresis

Immunofixation

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13
Q

What is a Bence-Jones protein?

A

Kappa free immunoglobulin light chain

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14
Q

Where are Bence-Jones proteins found in myeloma?

A

Urine

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15
Q

Outline the pathogenesis in multiple myeloma

A

Uncontrolled overproduction of one immunoglobulin which pushes out other healthy cells from the bone marrow

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16
Q

What is a paraprotein?

A

Abnormal immunoglobulin light chain produced by abnormal immunoglobulin

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17
Q

Why are lytic lesions found in myeloma?

A

Abnormal immunoglobulins activate osteoclasts through IL-6

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18
Q

What is the most common myeloma?

A

IgG

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19
Q

What is the second most common myeloma?

A

IgA

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20
Q

How does myeloma present?

A 
HyperCalcaemia
Renal failure
Anaemia
Bone pain
(CRAB)
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21
Q

How does the myeloma-induced renal failure present?

A

N%V
Pruritus and muscle cramps
Dyspnoea and oedema

22
Q

What causes nausea and vomiting in renal failure?

A

Uraemia

23
Q

What causes pruritus and muscle cramps in renal failure?

A

Increased phosphate

24
Q

What causes dyspnoea and oedema in renal failure?

A

Fluid retention

25
Q

Describe the anaemia in myeloma?

A

Normocytic normochromic

26
Q

Describe the bone pain in myeloma

A

Worse on activity

Common in spine and ribs

27
Q

What causes confusion, weakness and fatigue in myeloma?

A

Hypercalcaemia

28
Q

What causes headache and visual disturbance in myeloma?

A

Hyper viscosity

29
Q

What causes peripheral neuropathy in myeloma?

A

Amyloid deposition

30
Q

What do U&Es show in myeloma?

A

Raised CK

Hypercalcaemia

31
Q

What does blood film show in myeloma?

A

Rouleaux

32
Q

How is myeloma status assessed?

A

Bone marrow biopsy
Immunohistochemistry
Cytogenetics
Skeletal survey (MRI)

33
Q

What is the criteria for symptomatic myeloma?

A

Clonal plasma cells >10% of biopsy
Paraprotein in serum or urine
End-organ damage

34
Q

What is the criteria for asymptomatic myeloma?

A

Clonal plasma cells >10% biopsy
Serum paraprotein >30g/L
No myeloma related end organ damage

35
Q

How is asymptomatic myeloma treated?

A

Watchful waiting

36
Q

What is the MGUS criteria?

A

Serum paraprotein <30g/l
Clonal plasma cells <10% on bone marrow biopsy
No myeloma end organ damage

37
Q

How is myeloma treated?

A

<65 - combination chemo and autologous stem cell transplant

>65 - combination chemo

38
Q

What is “maintenance treatment” for myeloma?

A

Low dose chemo on a regular basis

39
Q

What symptomatic relief is given in myeloma?

A

Analgesia - not NSAID
Local radio - for cord comp
Bisphosphonates - bone pain
Vertebroplasty - stabilise vertebra

40
Q

What drugs are used with chemo in myeloma treatment?

A

Corticosteroids, dexmet, prednisolone
Alkylating agents
Thalidomide
Lenalidomide

41
Q

Give examples on an alkylating agent

A

Cylcophosphamide

Melphalan

42
Q

What is AL amyloidosis?

A

Mutation in the light chain of antibodies so they can’t be broken down
Accumulate in tissue as insoluble beta pleated sheet

43
Q

How is AL amyloidosis treated?

A

Chemo to switch off light chain supply

44
Q

What does biopsy and stain show in AL amyloidosis?

A

Congo red stain

Apple-green birefringence under polarised light

45
Q

Which investigations can be used to show amyloid deposition in other organs?

A

Serum amyloid scintigraphy scan
Echo
Nephrotic range proteinuria

46
Q

Which immunoglobulin is involved in Waldenstrom’s macroglobulinaemia?

A

IgM

47
Q

What is Waldenstrom’s Macroglobulinaemia?

A

Clonal disorder of cells resulting in a lymphoplasmacytoid neoplasm

48
Q

Why does Waldenstrom’s Macroglobulinaemia cause a greater degree of hyperviscosity than myeloma?

A

IgM has more binding sites

49
Q

How does hyperviscosity syndrome present?

A 
Fatigue
Visual problems
Confusion/coma
Bleeding
Cardaic failure
50
Q

How is Waldenstrom’s Macroglobulinaemia treated?

A

Plasmapheresis to physically remove IgM

Chemo

Year 3 - Haematology (17 decks)

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