Myeloma and other plasma cell

Question 1

Where and how are B cells Made

Answer 1

Bone marrow

From pluripotent haematopoietic stem cells

Question 2

What branch of the immune system are B cells in?

Answer 2

Adaptivee

Question 3

What are the 2 roles of B cells?

Answer 3

Antibody production

Antigen presenting cells

Question 4

What are immunoglobulins?

Answer 4

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

Question 5

Which immunoglobulins are monomers?

Answer 5

IgD
IgE
IgG

Question 6

Which immunoglobulins are Dimers?

Answer 6

IgA

Question 7

Which immunoglobulins are Pentamers?

Answer 7

IgM

Question 8

What cells are removed?

Answer 8

Self-reactive

Question 9

What B cells leave bone marrow?

Answer 9

Immature B cells with Immunoglobulin (Ig) on their surface

Question 10

What is the normal B lymphocyte maturation?

Answer 10

Stem cells
Lymphoid progenitors
Pro B cells
Pre B cells
IgM B cells

Question 11

Where do B cells travel to?

Answer 11

Follicle germinal centre of the lymph node

Question 12

What may B cells return to the marrow as?

Answer 12

Plasma cell

Question 13

What may B cells circulate as?

Answer 13

Memory B Cell

Question 14

What is a plasma cell?

Answer 14

Factory cell that produced antibody

Question 15

What are the characteristics of a plasma cell?

Answer 15

Open chromatin
Blue cytoplasm
Pale perinuclear area

Question 16

What are Polyconal immunoglobins reactive to?

Answer 16

Infection
Autoimmune
Malignancy
Liver disease

Question 17

What can create a polyclonal increase in immunoglobins?

Answer 17

Produced by many different plasma cell clones

Question 18

What causes a monoclonal rise in immunoglobulins?

Answer 18

All derived from clonal expansion of a single B-cell

Question 19

What does Monoclonal immunoglubin =?

Answer 19

Paraprotein

Question 20

What are monoclonal rise in immunolobulins a sign of?

Answer 20

An underlying clonal B-cell disorder

Question 21

How are immunoglobulins detected?

Answer 21

Serum electrophoresis

Question 22

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

Answer 22

0.5g/day

Question 23

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

Answer 23

BJP

Question 24

What types of light chains are there?

Answer 24

Kappa

Lambda

Question 25

What are causes of paraproteinaemia?

Answer 25

``` MGUS? Myeloma Amyloidosis Lymphoma Plasmacytoma Leukaemia ```

Question 26

What is myeloma?

Answer 26

A plasma cell malignancy

Question 27

What is the development of myeloma?

Answer 27

Normal plasma cells MGUS clone Asymptomatic myeloma Myeloma

Question 28

What is benign myeloma?

Answer 28

MGUS clone

Question 29

What is a malignant but non-organ damaging myeloma?

Answer 29

Asymptomatic myeloma

Question 30

What is an overtly malignancy myeloma?

Answer 30

Myeloma

Question 31

What 2 ways can myeloma affect the body?

Answer 31

Direct tumour cell effects | Paraprotein mediated effects

Question 32

What are direct tumour cell effects of myeloma?

Answer 32

Bone lesions Increased calcium Bone pain Marrow failure

Question 33

What are paraprotein mediated effects of myeloma?

Answer 33

Renal failure Immune suppresion Hyperviscosity Amyloid

Question 34

What is myeloma classified by?

Answer 34

Type of antibody produced

Question 35

What us the most common type of myeloma?

Answer 35

IgG

Question 36

What is lytic bone disease?

Answer 36

Multiple punched out lytic lesions in the skull/bones | Wedge compression fracture

Question 37

Why does lytic bone disease occur in myeloma?

Answer 37

IL-6 suppresses osteoblasts and activates osteoclasts

Question 38

What are the symptoms of hypercalcaemia?

Answer 38

``` Stones Bones Abdominal groans Psychiatric moans Thirst Dehydration Renal impairment ```

Question 39

What % of myeloma patients have renal impairment at diagnosis?

Answer 39

30%

Question 40

What type of renal impairment can occur with myeloma?

Answer 40

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

Question 41

What is the treatment of cast nephropathy?

Answer 41

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

Question 42

What is the average age of diagnosis of myeloma?

Answer 42

65

Question 43

How is myeloma treated?

Answer 43

Chemotherapy Steroids Alkylating agents Stem cell transplant

Question 44

What level may monitor response to myeloma treatment?

Answer 44

Paraprotein level

Question 45

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

Answer 45

Daratumumab

Question 46

Does myeloma always relapse?

Answer 46

Yes

Question 47

How are myeloma symptoms controlled?

Answer 47

Opiate Radiotherapy Bisphosphonates Vertebroplasty

Question 48

What is MGUS?

Answer 48

Monoclonal gammopathy of undetermined significance

Question 49

What is the definition of MGUS/

Answer 49

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

Question 50

What is evidence of myeloma end organ damage?

Answer 50

``` Abnormal calcium Abnormal renal function Abnormal Hb Lytic lesions Increase in infection ```

Question 51

Which gender is more affected by MGUS?

Answer 51

Men

Question 52

What is AL amyloidosis?

Answer 52

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

Question 53

What does accumulation of amyloid in tissues cause?

Answer 53

organ damage

Question 54

What is AL amyloidosis like?

Answer 54

Slowly progressive | Multisystem

Question 55

What is the treatment of AL amyloidosis?

Answer 55

chemo to switch off light chain supply

Question 56

What kidney damage occurs in AL amyloid?

Answer 56

Nephrotic syndrome

Question 57

What heart damage occurs in AL amyloid?

Answer 57

Cardiomyopathy

Question 58

What liver damage occurs in AL amyloid?

Answer 58

Organomegaly deranged LFTs

Question 59

What neuropathy occurs in AL amyloid?

Answer 59

Autonomic | PeripheraL

Question 60

What GI tract problems occur in AL?

Answer 60

Malabsorption

Question 61

What stain is done for amyloid dianosis?

Answer 61

Congo red stain | Rectal or fat biopsy

Question 62

What scan can be used to monitor AL amyloid?

Answer 62

SAP scan

Question 63

What does AL amyloid do in polarised light?

Answer 63

Apple green birefringence

Question 64

What is Waldenstrom's macroglobulinaemia?

Answer 64

Lymphoplasmacytoid neoplasm

Question 65

What are the tumour effects of WM?

Answer 65

Lymphadenopathy Splenomegaly Marrow failure

Question 66

What re the Paraprotein effects?

Answer 66

Hyperviscosity | Neuropathy

Question 67

What are the clincal features of Hyperviscosity syndrome?

Answer 67

``` Fatigue Visual disturbance Confusion Coma Bleeding Cardiac failure ```

Question 68

What are B symtpoms?

Answer 68

Night sweats | Weight Loss

Question 69

What is the treatment of waldenstroms?

Answer 69

Chemotherapy | Plasmapheresis