Multiple myeloma/amyloidosis Flashcards
Define multiple myeloma
Neoplasm of plasma cells leading to a production of monoclonal immunoglobulin paraprotein
Epidemiology of multiple myeloma
MIddle aged to elderly
Symptoms of MM
CRAB
HIGH calcium
RENAL FAILURE - nephrotic syndrome
Anaemia + pancytopenia
Bones - pain, osteoporosis, osteolytic lesions, fractures
Hyperviscosity syndrome
Inv + what would be seen
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
Staging system for multiple myeloma
Durie-Salmon staging system
Mx for MM
CRAB symptom support
Bisphosphonates
Name some first line MM drugs
Bortezomib +/- the following:
Dexamethasone
Cyclophosphamide
Lenalidomide
When in remission, mx
Auto-SCT
If not suitable for SCT
Dara-tu-mu-mab (anti-CD38)
Car-fil-zomb ) (protease inhibitors)
Mnemonic
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)
What other diseases can be picked up when screening for multiple myeloma
Monoclonal gammopathy of unknown significance
Smouldering MM
What marker is used to identify MGUS, s-MM and MM?
M spike on the serum electrophoresis and bone marrow plasma cell concentration
Differentiate between MGUS, s-MM and MM
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
Which of MGUS and s-MM can transform to MM? Which needs treatment?
Neither needs treating, but s-MM can become mm
What other conditions can result from amyloidosis/cause it?
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
Clinical clues of amyloidosis
Hepatomegaly
HF
Macroglossia
Histology for amyloidosis
AMY does the CONGO and gets a GREEN APPLE
Apple green birefringence on CONGO RED STAIN
On the topic of restrictive cardiomyopathy, what other things cause this?
Sarcoidosis
Amyloidosis
Radiotherapy induced fibrosis
What other types of cardiomyopathy are there?
Dilated
Hypertrophic
What causes dilated cardiomyopathy
Alcohol
Idiopathic
Thyroid disease
Viral
Haemochromatosis
What is amylodosis
Abnormal folding of amyloid leading to deposition in tissues, causing disruption of function.
Types:
Beta pleated sheet structure
Resistant to enzyme degradation
Primary vs secondary amyloidosis
Primary = Multiple myeloma / AL amyloidosis
Deposition of monoclonal Ig light chains; BJP in serum and urine
Secondary = acute phase protein amyloid (AA)
Causes of secondary amyloidosis
Chronic inflammation - RA, IBD, Bronchiectasis, Familial Mediterranean fever
Chronic infection - TB osteomyelitis, IVDU
Non immune - renal cell carcinoma, Hodgkin’s
Symptoms of amyloidosis + why
Symptoms largely due to where it is deposited:
Nephrotic syndrome - most common presentation
Macroglossia
Hepatosplenomegaly
Restrictive cardiomyopathy, heart failure, conduction defects
Carpal tunnel
Carpal tunnel amyloidosis is associated with what cause and what kind of amyloid deposition?
Haemodialysis
Beta 2 amyloid
Histology stain for amyloidosis
Congo red stain - apple green birefringence
