MYELOMA + some extra cheeky diseases

Question 1

what is myeloma?

Answer 1

Cancer of the plasma cells causing them to produce paraproteins (monoclonal antibodies)

Question 2

presentation of myeloma?

Answer 2

CRAB
-hypercalcaemia
-renal impairement
-anaemia
-bone pain

Question 3

why does myeloma cause renal impairement?

Answer 3

Para proteins cause cast nephropathy- clog up kidney filtration system

Question 4

why is there hypercalcaemia in myeloma?

Answer 4

-due to increase of osteoclast activity and surpressed osteoblast activity (more bone being broken down than being made)
-increase in osteoclast activity causes a lot of calcium to be absorbed from the bone into blood increasing calcium

Question 5

what occurs to plasma viscosity and why- myeloma?

Answer 5

-plasma viscosity increase when there are more proteins in blood (paraprotein)

Question 6

how is myeloma classified and give some examples

Answer 6

Classified by type of antibody produced:
-IgG (most common)
-Bence jones myeloma
-IgA
-IgE
-Biclonal

Question 7

what type of antibody increases plasma viscosity the most and why?

Answer 7

IgM
-because its a pentamere!

(associated with WALDENSTROM MACROGLOBULINAEMIA)

Question 8

top 3 most common types of myeloma?

Answer 8

-IgG (most common)
-IgA (2nd)
-Bence jones myeloma (free light chain)

Question 9

what are antibodies made up of?

Answer 9

2 heavy chains and 2 light chains

Question 10

blood film of someone with myeloma?

Answer 10

rouleaux formation (stacking of RBC)

Question 11

what can be found in urine of someone with certain type of myeloma?

Answer 11

BENCE JONES PROTEINS

Question 12

Imagine of choice for myeloma?

Answer 12

full body MRI!
-will allow you to assess bone lesions (so lytic!)

Question 13

X-ray findings on myeloma?

Answer 13

-punched out lesions
-lytic lesions

Question 14

usual management of myeloma?

Answer 14

usually patients quite old so less aggressive treatment= combination chemotherapy

Question 15

management of myeloma in young/ fit patients?

Answer 15

high dose chemo/ autologous stem cell transplant

Question 16

what is used to manage hypercalcaemia + bone pain?

Answer 16

biphosphonates

Question 17

what is used to fix fractures bones e.g. vertebrae?

Answer 17

Vertebroplasty
- inject sterile cement into fractured bone to stabilise

Question 18

what is MGUS?

Answer 18

-benign myeloma precursor

-when there is an excess of a single type of antibody or antibody components without other features of myeloma or cancer

Question 19

how likely are you to get myeloma with MGUS?

Answer 19

-slightly increased risk of developing myeloma (increased by 1% every year)

most people actually die of old age not myeloma

Question 20

findings- MGUS? (monoglonal gammopathy of undertermined significance)

Answer 20

Paraprotein <30g/l
Bone marrow plasma cells <10%

No evidence of myeloma end organ damage:
-normal calcium
-normal renal function
-normal haemoglobin
-no lytic lesions
-no increase in infections

Question 21

what is AL amyloidosis?

Answer 21

Rare, benign disorder that is similar to MGUS

-There is a mutation in the light chains of the antibodies produced by the plasma cells, this alters their structure so they are insoluble beta pleated sheets
-these insoluble beta sheets/ amyloid cause end organ damage

Question 22

how does AL amyloidosis present?

Answer 22

Most commonly= weakness and dyspnoea

Depends what organ is effected

(2/3rds) Kidney involvement:
-nephrotic syndrome

(1/2) Heart involvement:
-Cardiomyopathy

Question 23

BUZZ WORDY INVESTIGATION!! to confirm AL amyloidosis

Answer 23

Gold standard =
Organ biopsy

+

Congo red staining- will show ‘Apple green’ birefringement under polarised light

Question 24

what type of biopsy can be done that is less invasive for AL amyloidosis?

Answer 24

rectal or fat biopsy

Question 25

apart from biopsy- what other investigations can be done for AL amyloidosis?

Answer 25

-SAP scan
-Echo/ cardiac MRI
-Nephrotic range proteinuria

Question 26

treatment + prognosis of AL amyloidosis?

Answer 26

CHEMO!!
-poor prognosis especially if cardiac amyloid :(

Question 27

What is Waldenstroms macroglobinuria?

Answer 27

Chronic, indolent, B-cell lymphoproliferative disorder characterised by an IgM monoclonal paraprotein and bone marrow infiltration by lymphoplasmacytic cells

Question 28

what type of antibody is produced by Waldenstroms macroglobinuria and what effect does this have?

Answer 28

IgM
-IgM is a pentmere so significantly increases PV

Question 29

presentation of Waldenstroms macroglobinuria?

Answer 29

Hyperviscosity syndrome :
-Fatigue, visual disturbance, confusion, coma
-Bleeding
-Cardiac failure

B symptoms; night sweats, weight loss

Question 30

symptoms of hyperviscosity syndrome?

Answer 30

-Fatigue, visual disturbance, confusion, coma
-Bleeding
-Cardiac failure

Question 31

treatment- waldenstroms macroglobinuria?

Answer 31

-Chemo
-Plasmapheresis (as you need to reduce the viscosity of the blood)