Multiple myeloma/amyloidosis

Question 1

Define multiple myeloma

Answer 1

Neoplasm of plasma cells leading to a production of monoclonal immunoglobulin paraprotein

Question 2

Epidemiology of multiple myeloma

Answer 2

MIddle aged to elderly

Question 3

Symptoms of MM

Answer 3

CRAB
HIGH calcium
RENAL FAILURE - nephrotic syndrome
Anaemia + pancytopenia
Bones - pain, osteoporosis, osteolytic lesions, fractures
Hyperviscosity syndrome

Question 4

Inv + what would be seen

Answer 4

Serum electrophoresis - dense narrow band in gamma region (monoclonal); identified according to Ig type, then as kappa or lambda

Blood film - rouleux (RBC stacking)
- bone profile to check for calcium
U&E for renal function
FBC for anaemia
ESR

CT / MRI for boney lesions

Urine - BJP

Question 5

Staging system for multiple myeloma

Answer 5

Durie-Salmon staging system

Question 6

Mx for MM

Answer 6

CRAB symptom support
Bisphosphonates

Question 7

Name some first line MM drugs

Answer 7

Bortezomib +/- the following:
Dexamethasone
Cyclophosphamide
Lenalidomide

Question 8

When in remission, mx

Answer 8

Auto-SCT

Question 9

If not suitable for SCT

Answer 9

Dara-tu-mu-mab (anti-CD38)
Car-fil-zomb ) (protease inhibitors)

Question 10

Mnemonic

Answer 10

Because I was on DURIE duty for MULTIPLE days, I was so bored and hungry so went to buy SALMON.

(Durie-salmon staging for multiple myeloma.)

I BORTE CRAB instead.

(Calcium high, renal failure nephrotic syndrome, anaemia, bone pain.)

And it bit me, so I BORTE ZOM IBuprofen.

(First line drug = bortezomib.)

As I couldn’t SC(a)T(e) to the jury place

(When in remission, auto-stem cell transplant)

My friend DARA TUk MUm’s CAR to FIL it with ZOM PROTEIN petrol so I could drive there instead.

(If SCT not applicable, daratumumab (anti-CD38) or carfilzomb = protease inhibitors)

Question 11

What other diseases can be picked up when screening for multiple myeloma

Answer 11

Monoclonal gammopathy of unknown significance
Smouldering MM

Question 12

What marker is used to identify MGUS, s-MM and MM?

Answer 12

M spike on the serum electrophoresis and bone marrow plasma cell concentration

Question 13

Differentiate between MGUS, s-MM and MM

Answer 13

MGUS
M-spike is less than 30g/l and the BM is <10 clonal plasma cells

s-MM
M-spike more than 30g/L, BM is more than 10 percent clonal plasma cells

MM - M spike more than 30g/L and BM more than 60 percent clonal plasma cells / any clonal population

Question 14

Which of MGUS and s-MM can transform to MM? Which needs treatment?

Answer 14

Neither needs treating, but s-MM can become mm

Question 15

What other conditions can result from amyloidosis/cause it?

Answer 15

Causes:
chronic inflammation causes AA amyloidosis (amyloid A)
E.g. rheumatic arthritis, chronic infections, bronchiectasis (inflammation = raised acute phase protein produced by liver)

Amyloid L (AL) - multiple myeloma

Amyloidosis can cause:
Restrictive cardiomyopathy
Nephrotic syndrome

Question 16

Clinical clues of amyloidosis

Answer 16

Hepatomegaly
HF
Macroglossia

Question 17

Histology for amyloidosis

Answer 17

AMY does the CONGO and gets a GREEN APPLE

Apple green birefringence on CONGO RED STAIN

Question 18

On the topic of restrictive cardiomyopathy, what other things cause this?

Answer 18

Sarcoidosis
Amyloidosis
Radiotherapy induced fibrosis

Question 19

What other types of cardiomyopathy are there?

Answer 19

Dilated
Hypertrophic

Question 20

What causes dilated cardiomyopathy

Answer 20

Alcohol
Idiopathic
Thyroid disease
Viral
Haemochromatosis

Question 21

What is amylodosis

Answer 21

Abnormal folding of amyloid leading to deposition in tissues, causing disruption of function.

Types:
Beta pleated sheet structure
Resistant to enzyme degradation

Question 22

Primary vs secondary amyloidosis

Answer 22

Primary = Multiple myeloma / AL amyloidosis
Deposition of monoclonal Ig light chains; BJP in serum and urine

Secondary = acute phase protein amyloid (AA)

Question 23

Causes of secondary amyloidosis

Answer 23

Chronic inflammation - RA, IBD, Bronchiectasis, Familial Mediterranean fever
Chronic infection - TB osteomyelitis, IVDU
Non immune - renal cell carcinoma, Hodgkin’s

Question 24

Symptoms of amyloidosis + why

Answer 24

Symptoms largely due to where it is deposited:

Nephrotic syndrome - most common presentation
Macroglossia
Hepatosplenomegaly
Restrictive cardiomyopathy, heart failure, conduction defects
Carpal tunnel

Question 25

Carpal tunnel amyloidosis is associated with what cause and what kind of amyloid deposition?

Answer 25

Haemodialysis
Beta 2 amyloid

Question 26

Histology stain for amyloidosis

Answer 26

Congo red stain - apple green birefringence