Myeloma and other plasma cell Flashcards

1
Q

Where and how are B cells Made

A

Bone marrow

From pluripotent haematopoietic stem cells

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2
Q

What branch of the immune system are B cells in?

A

Adaptivee

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3
Q

What are the 2 roles of B cells?

A

Antibody production

Antigen presenting cells

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4
Q

What are immunoglobulins?

A

Antibodies produced by B cells and Plasma cells
Proteins
2 Heavy 2 light

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5
Q

Which immunoglobulins are monomers?

A

IgD
IgE
IgG

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6
Q

Which immunoglobulins are Dimers?

A

IgA

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7
Q

Which immunoglobulins are Pentamers?

A

IgM

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8
Q

What cells are removed?

A

Self-reactive

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9
Q

What B cells leave bone marrow?

A

Immature B cells with Immunoglobulin (Ig) on their surface

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10
Q

What is the normal B lymphocyte maturation?

A
Stem cells
Lymphoid progenitors
Pro B cells
Pre B cells
IgM B cells
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11
Q

Where do B cells travel to?

A

Follicle germinal centre of the lymph node

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12
Q

What may B cells return to the marrow as?

A

Plasma cell

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13
Q

What may B cells circulate as?

A

Memory B Cell

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14
Q

What is a plasma cell?

A

Factory cell that produced antibody

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15
Q

What are the characteristics of a plasma cell?

A

Open chromatin
Blue cytoplasm
Pale perinuclear area

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16
Q

What are Polyconal immunoglobins reactive to?

A

Infection
Autoimmune
Malignancy
Liver disease

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17
Q

What can create a polyclonal increase in immunoglobins?

A

Produced by many different plasma cell clones

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18
Q

What causes a monoclonal rise in immunoglobulins?

A

All derived from clonal expansion of a single B-cell

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19
Q

What does Monoclonal immunoglubin =?

A

Paraprotein

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20
Q

What are monoclonal rise in immunolobulins a sign of?

A

An underlying clonal B-cell disorder

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21
Q

How are immunoglobulins detected?

A

Serum electrophoresis

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22
Q

How much free light chain in produced by normal plasma cells?

A

0.5g/day

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23
Q

What can excess free light chains leak into the urine as?

A

BJP

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24
Q

What types of light chains are there?

A

Kappa

Lambda

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25
Q

What are causes of paraproteinaemia?

A
MGUS?
Myeloma
Amyloidosis
Lymphoma
Plasmacytoma
Leukaemia
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26
Q

What is myeloma?

A

A plasma cell malignancy

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27
Q

What is the development of myeloma?

A

Normal plasma cells
MGUS clone
Asymptomatic myeloma
Myeloma

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28
Q

What is benign myeloma?

A

MGUS clone

29
Q

What is a malignant but non-organ damaging myeloma?

A

Asymptomatic myeloma

30
Q

What is an overtly malignancy myeloma?

A

Myeloma

31
Q

What 2 ways can myeloma affect the body?

A

Direct tumour cell effects

Paraprotein mediated effects

32
Q

What are direct tumour cell effects of myeloma?

A

Bone lesions
Increased calcium
Bone pain
Marrow failure

33
Q

What are paraprotein mediated effects of myeloma?

A

Renal failure
Immune suppresion
Hyperviscosity
Amyloid

34
Q

What is myeloma classified by?

A

Type of antibody produced

35
Q

What us the most common type of myeloma?

A

IgG

36
Q

What is lytic bone disease?

A

Multiple punched out lytic lesions in the skull/bones

Wedge compression fracture

37
Q

Why does lytic bone disease occur in myeloma?

A

IL-6 suppresses osteoblasts and activates osteoclasts

38
Q

What are the symptoms of hypercalcaemia?

A
Stones
Bones
Abdominal groans
Psychiatric moans
Thirst
Dehydration
Renal impairment
39
Q

What % of myeloma patients have renal impairment at diagnosis?

A

30%

40
Q

What type of renal impairment can occur with myeloma?

A
Tubular cell damage bby light chains
Cast Nephropathy
Sepsis
Hypercalcaemia
Dehydration
Drugs (NSAIDS)
Hyperuricaemia
Amyloid
41
Q

What is the treatment of cast nephropathy?

A
Damage may be reversible with prompt treatment
Hydration 
Stop nephrotoxic drugs
Switch off light chian production
-Steroids/Chemo
42
Q

What is the average age of diagnosis of myeloma?

A

65

43
Q

How is myeloma treated?

A

Chemotherapy
Steroids
Alkylating agents
Stem cell transplant

44
Q

What level may monitor response to myeloma treatment?

A

Paraprotein level

45
Q

What is an example of a monoclonal antibody therpay for myeloma?

A

Daratumumab

46
Q

Does myeloma always relapse?

A

Yes

47
Q

How are myeloma symptoms controlled?

A

Opiate
Radiotherapy
Bisphosphonates
Vertebroplasty

48
Q

What is MGUS?

A

Monoclonal gammopathy of undetermined significance

49
Q

What is the definition of MGUS/

A

Paraprotein <30g/l
Bone marrow plasma <10%
No evidence of myeloma end organ damage

50
Q

What is evidence of myeloma end organ damage?

A
Abnormal calcium
Abnormal renal function
Abnormal Hb
Lytic lesions
Increase in infection
51
Q

Which gender is more affected by MGUS?

A

Men

52
Q

What is AL amyloidosis?

A

Rare disorder
Small plasma cell clone
Mutation in light chain
Precipitates in tissues as insoluble beta pleated sheet

53
Q

What does accumulation of amyloid in tissues cause?

A

organ damage

54
Q

What is AL amyloidosis like?

A

Slowly progressive

Multisystem

55
Q

What is the treatment of AL amyloidosis?

A

chemo to switch off light chain supply

56
Q

What kidney damage occurs in AL amyloid?

A

Nephrotic syndrome

57
Q

What heart damage occurs in AL amyloid?

A

Cardiomyopathy

58
Q

What liver damage occurs in AL amyloid?

A

Organomegaly deranged LFTs

59
Q

What neuropathy occurs in AL amyloid?

A

Autonomic

PeripheraL

60
Q

What GI tract problems occur in AL?

A

Malabsorption

61
Q

What stain is done for amyloid dianosis?

A

Congo red stain

Rectal or fat biopsy

62
Q

What scan can be used to monitor AL amyloid?

A

SAP scan

63
Q

What does AL amyloid do in polarised light?

A

Apple green birefringence

64
Q

What is Waldenstrom’s macroglobulinaemia?

A

Lymphoplasmacytoid neoplasm

65
Q

What are the tumour effects of WM?

A

Lymphadenopathy
Splenomegaly
Marrow failure

66
Q

What re the Paraprotein effects?

A

Hyperviscosity

Neuropathy

67
Q

What are the clincal features of Hyperviscosity syndrome?

A
Fatigue
Visual disturbance
Confusion
Coma
Bleeding
Cardiac failure
68
Q

What are B symtpoms?

A

Night sweats

Weight Loss

69
Q

What is the treatment of waldenstroms?

A

Chemotherapy

Plasmapheresis