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Y3 - Haem > Paraprotein Disease > Flashcards

Paraprotein Disease Flashcards

1
Q

What are plasma cells?

A

Antibody factories that produce lots of antibodies in response to antigen presentation

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2
Q

What do plasma cells look like?

A

Open chromatin
Blue cytoplasm
Pale perinuclear area
Look like blue fried eggs

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3
Q

What is the structure of antibodies?

A

2 heavy chains
2 light chains
Constant region
Variable region

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4
Q

What are the types of light chain?

A

Kappa

Lambda

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5
Q

What is the purpose of the variable region in antibodies?

A

Allows specificity in binding to antigens

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6
Q

What are free circulating light chains?

A

Production of light chains is slightly higher than production of heavy chains, so some light chains circulate freely in the body

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7
Q

What is the difference between polyclonal and monoclonal expansion?

A

Polyclonal is the expansion of a variety of B cell populations
Monoclonal is the expansion of a single B cell population

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8
Q

What is monoclonal expansion known as?

A

Paraprotein disease

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9
Q

What situations does polyclonal expansion occur in?

A

Infection
Malignancy
Autoimmune disease

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10
Q

What is the immunoglobulin produced excessively in monoclonal expansion known as?

A

A paraprotein

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11
Q

What are causes of paraprotein disease?

A 
Myeloma
MGUS
AL amyloidosis
Waldenstrom's macroglobinaemia
B cell lymphoma
Chronic lymphocytic leukaemia
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12
Q

What will paraprotein disease show on blood film, and what does this look like?

A

Rouleaux formation

Stacking of blood cells

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13
Q

What is myeloma?

A

Malignant proliferating fo plasma B cells in the bone marrow

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14
Q

How is myeloma classified?

A 
Based on the class of antibody and type of light chain produced
IgG, IgA
Kappa, lambda
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15
Q

What pathophysiological effect does myeloma have on bone?

A 
Breakdown of bone 
- erosion by up regulation of osteoclast activity and downregulation of osteoblasts
- formation of osteolytic lesions
- hypercalcaemia
Infiltration of marrow
- marrow failure
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16
Q

What pathophysiological effect does myeloma have due to antibody and light chain effects?

A

Increased production of antibodies
- hyperviscocity
- immunosuppression due to failure to produce normal antibodies
Glomerular disease
- deposition of light chains in the kidney causing cast nephropathy and decline in renal function
Amyloid

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17
Q

What is the median age at diagnosis of myeloma?

A

65-70

18
Q

What is the presentation of myeloma?

A 
Bone pain (back, worse at night)
Pathological fractures
Spinal cord compression
Hypercalcaemia - stones, bones, groans, thrones, psychiatric moans
Anaemia symptoms
Bleeding
Bruising
Recurrent infections
Reduced vision
Reduced cognition
19
Q

What are the symptoms of hypercalcaemia?

A

Stones - renal stones and other renal disease
Bones - bone pain
Groans - abdominal pain
Thrones - thirst, polydipsia, constipation
Psychiatric moans - depression, anxiety

20
Q

What causes the reduced vision and cognition in myeloma?

A

Hyperviscocity

21
Q

What do bloods show in myeloma?

A

Raised ESR
U&Es - reduced renal function
Hypercalcaemia
Normochromic normocytic anaemia

22
Q

How is myeloma diagnosed?

A

Serum electrophoresis showing paraprotein

Diagnosis confirmed on bone marrow aspirate and trephine biopsy

23
Q

What can be seen by urine electrophoresis in myeloma?

A

Bence Jones protein

24
Q

What can be seen on skeletal survey in myeloma?

A

Pepper-pot skull

Vertebral collapse

25
Q

What are management options in myeloma?

A 
Dexamethasone
Thalidomide
Melphalan, cyclophosphamide
Monoclonal antibodies - daratumumab
High dose chemotherapy/autologous stem cell transplant (AHSCT) in fit patients
26
Q

What is used to monitor response to myeloma treatment?

A

Paraprotein

27
Q

What is used for symptomatic control of bone pain in myeloma?

A

Analgesia (opiates - avoid NSAIDs)

Bisphosphonates

28
Q

What is monoclonal homeopathy of unknown significance (MGUS)?

A

Paraproteinaemia without pathological consequences

Can progress to myeloma

29
Q

What is the definition of MGUS?

A

<30g paraprotein
Bone marrow plasma cells <10%
No evidence of myeloma or end organ damage

30
Q

What tests need to be done to exclude myeloma or end organ damage to diagnose MGUS?

A 
Calcium
Hb
U&Es
Bone scans
Make sure no increase in systemic infections
31
Q

What is the presentation of MGUS?

A

Usually asymptomatic

10-30% have demyelinating neuropathy

32
Q

What is AL Amyloidosis?

A

Group of disorders characterised by production of abnormal light chains from B cell clonal population

33
Q

What is the pathophysiology of AL amyloidosis?

A

Abnormal light chains precipitate into tissues and form insoluble beta-amyloid sheets
The amyloid sheets develop in multiple organs and cause multiple end organ damage
Can be primary or secondary to myeloma or Waldenstrom’s

34
Q

What is the disease pattern of AL amyloidosis?

A

Slowly progressive

Multisystem disease

35
Q

What is the presentation of AL amyloidosis?

A 
Nephotic syndrome
Hepatosplenomegaly
Macroglossia
Malabsorption
Restrictive cardiomyopathy, sudden death
Autonomic and peripheral neuropathy
Periorbital purpura (characteristic)
36
Q

What is a diagnostic sign of AL amyloidosis?

A

Apple green birefringence with congo red stain on organ biopsy of affected tissue or rectum/fat

37
Q

What do blood tests show in AL amyloidosis?

A

Deranged LFTs, U&Es, etc

38
Q

What is the management of AL amyloidosis?

A

Chemotherapy to attempt to switch of light chain supply

39
Q

What is Waldenstrom’s macroglobinaemia?

A

IgM paraproteinaemia caused by expansion of a cell population intermediate between a plasma cell and lymphocyte - lymphoplasmacytoid neoplasm

40
Q

What is the presentation of Waldenstrom’s macroglobinaemia?

A 
Hyperviscocity
- fatigue
- confusion
- reduced cognition
- visual disturbances
- chest and abdominal pain
B symptoms
- weight loss
- night sweats
- splenomegaly
- lymphadenopathy
41
Q

What do investigations show in Waldenstrom’s macroglobinopathy?

A

Raised ESR

IgM paraproteinaemia on electrophoresis

42
Q

What is the management of Waldenstrom’s macroglobinopathy?

A

Chemotherapy - chlorambucil

Hyperviscocity - leukapheresis or plasmapheresis

Y3 - Haem (9 decks)

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