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HAEMATOLOGY > Myeloma and Plasma Cell Dyscrasias > Flashcards

Myeloma and Plasma Cell Dyscrasias Flashcards

1
Q

Are B cells part of the adaptive or innate immune system response?

A

Adaptive

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2
Q

What are the main functions of B cells?

A

Make antibodies

- can also act as antigen presenting cells

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3
Q

Describe the structure of the Antibodies produced by B cells?

A

2 heavy chains (denoted by greek letters, but indicate type of Ab => M,G,D,A,E)

2 light chains (kappa or lambda)

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4
Q

What type of antibody can form a dimer?

A

IgA

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5
Q

What type of antibody can form a pentamer?

A

IgM

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6
Q

Why do antibodies have variable regions?

A

To change their structure in order to bind different antigens
- VDJ proteins are rearranged in order to do this

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7
Q

Describe the Precursors of the B cell, starting with a haematopoietic stem cell

A 
Stem cell
lymphoid progenitor
Pro B cell (can make IgM)
Pre B cell (can make IgM and IgD)
**then leave bone marrow and enter circulation**
B cell can then mature to plasma cell
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8
Q

B cells have to undergo class switching before maturing to a plasma cell. TRUE/FALSE?

A

TRUE

- as plasma cell can only make one type of Ab

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9
Q

Where do B cells travel to after they leave the bone marrow and what is their role here?

A
  • travel to follicle germinal centre in lymph node
  • identify antigen and improve fit
  • can return to marrow as plasma cell or circulate as memory B cell
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10
Q

Describe the appearance of a plasma cell on blood film

A
  • “eccentric” clock face nucleus with H+E staining
  • open chromatin nucleus
  • plentiful blue cytoplasm (lots of protein)
  • pale perinuclear area (golgi apparatus)
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11
Q

Describe the difference between a polyclonal and monoclonal expansion

A 
Polyclonal = increase in number of cells, but all are different
monoclonal = increase in cells but all are the same
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12
Q

What would cause a polyclonal rise in Ig (i.e. different types of Ab produced?

A
  • infection
  • autoimmune
  • malignancy (reaction of the host to non-haem malignancy in their body)
  • liver disease
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13
Q

What would cause a monoclonal rise in Ig?

A

clonal expansion of B cells (also called paraprotein)
identical Ab produced
marker of underlying clonal B cell disorder

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14
Q

How are Ig detected?

A

Serum electrophoresis

- detects abnormal protein bands (i.e. if there is too much of a specific protein)

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15
Q

How are the abnormal Ig in the blood classified and quantifed?

A

serum immunofixation

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16
Q

What is Bence Jones protein and where is it found?

A
  • light chains are found in the patients urine
  • can either be monomer light chain (kappa)
    OR can be dimer light chain (lambda)
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17
Q

What are the potential causes of paraproteinaemia?

A

MGUS - Monoclonal Gammaopathy of Unknown Significance
Myeloma
Amyloidosis
Lymphoma

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18
Q

What is MGUS?

A

Monoclonal expansion of a B cell creating a high number of certain Ig
- considered a benign/pre-malignant stage of Myeloma

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19
Q

What are the two ways in which myeloma can affect the body?

A

Direct tumour effects:

  • bone lesions and pain
  • increased calcium
  • marrow failure

Paraprotein effects

  • renal failure
  • immunosuppression
  • hyperviscosity
  • amyloid
20
Q

Describe how myeloma bone lesions normally appear on X-ray?

A

lytic bone disease
=> punched out lesions are common in the skull
=> wedge fractures in the spine = common

21
Q

What types of Ig are most commonly produced in myeloma?

A

IgG

IgA

22
Q

Why does lytic bone disease occur in myeloma?

A

myeloma cells increase IL-6 from marrow stromal cells

=> these activate osteoclasts to breakdown bone and release Ca2+

23
Q

What symptoms do patients often experience if they have hypercalcaemia?

A
  • stones
  • bones
  • abdominal groans
  • psychiatric moans
  • thirst
  • polyuria
  • dehydration
  • renal impairment
24
Q

Why do patients with myeloma get cast nephropathy if the kidneys can usually filter light chains?

A
  • proximal tubules usually reabsorb light chains
  • if overwhelmed they pass into loop of henle and combine with Tamm-Horsfall Protein in thick ascending limb
  • this compound then can block the nephron
25
Q

How is cast nephropathy treated?

A
  • switch off light chain production with steroids and chemotherapy
  • this may be able to reverse the damage
26
Q

What is the median age of myeloma diagnosis?

A

65 years

27
Q

How is myeloma treated?

A

Combination chemotherapy

  • steroids
  • alkylating agent (e.g. cyclophosphamide)
  • novel agents (e.g. thalidomide)
  • stem cell transplants also used in fit patients
28
Q

How can we monitor response to myeloma treatment?

A

Paraprotein level

29
Q

Describe what happens in an Autologous Haematopoietic stem cell transplant (AUSCT)?

A
  • pre treatment (releases stem cells from marrow)
  • collect stem cells and freeze in lab
  • administer chemo to wipe out immune system
  • return thawed blood stem cells
  • supportive tx for 4 weeks as immune system rebuilds
30
Q

What monoclonal antibody treatment can be used in myeloma?

A

Daratumab

- targets CD38

31
Q

What is prognosis like in myeloma?

A

5-10 years for younger patients

BUT relapse is inevitable

32
Q

How are myeloma symptoms controlled?

A
  • bone pain = opiate analgesia (NOT NSAIDs)
  • local radiotherapy
  • bisphosphonates
  • vertebroplasty
33
Q

How much paraprotein is found in the blood in an MGUS?

A

<30g/L

34
Q

What percentage of plasma cells are found in the blood in MGUS?

A

<10%

35
Q

MGUS causes no end organ damage. TRUE/FALSE?

A

TRUE

- normal Ca, renal function and Hb

36
Q

What gender has a higher prevalence of MGUS?

A

Males

37
Q

What is AL amyloidosis?

A

light chain mutation
causes accumulation of beta pleated sheets
these deposit in organs and cause damage

38
Q

What organs can be damaged by amyloid deposits?

A 
kidney
heart
liver
neuropathy
GI tract
39
Q

What treatment is used in amyloidosis?

A

Chemotherapy => similar to myeloma

40
Q

How is amyloidosis diagnosed/staged?

A
  • organ biopsy
  • congo red staining
  • shows apple green birefringence under light
41
Q

HOw can you check if amyloid has deposited in other organs?

A
  • SAP scan (full body scan with iodine contrast)
  • ECHO
  • proteinuria
42
Q

What is Waldenstrom’s Macroglobulinaemia?

A

IgM paraprotein disorder

- problem with maturation of B cell to plasma cell

43
Q

What direct effects are caused by the tumour in Waldenstrom’s Macroglobulinaemia

A
  • lymphadenopathy
  • splenomegaly
  • marrow failure
44
Q

What effects are caused by the raised IgM paraprotein in Waldenstrom’s Macroglobulinaemia?

A
  • hyperviscosity of blood
    => fatigue, visual disturbance, confusion, bleed, HF
  • neuropathy
45
Q

How is Waldenstrom’s Macroglobulinaemia treated?

A 
Chemo
plasma exchange (to remove pararprotein from circulation)

HAEMATOLOGY (24 decks)

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