Myeloma

Question 1

what is myeloma?

Answer 1

plasma cell malignancy

Question 2

cascade to myeloma

Answer 2

MGUS (benign/ premalignant)
asymptomatic myeloma (malignant but no organ damage)
myeloma

Question 3

how is myeloma classified?

Answer 3

according to type of antibody produced

Question 4

median age at diagnosis of myeloma

Answer 4

65

Question 5

direct tumour effects in myeloma

Answer 5

increased Ca2+, bone pain
bone marrow failure
lytic bone disease

Question 6

what does lytic bone disease involve?

Answer 6

vertebral compression factures

spinal cord compression due to osteoclasts/osteoblasts

Question 7

paraprotein effects in myeloma

Answer 7

renal failure
immune suppression
hyperviscosity
amyloid

Question 8

how can myeloma affect the kidneys?

Answer 8

tubular cell damage by light chains
cast nephropathy (Tamm-Horsfall protein)
indirectly

Question 9

how can myeloma indirectly affect the kidney

Answer 9

sepsis
hypercalcaemia + dehydration
NSAIDs used for pain
hyperuricaemia

Question 10

diagnosis of myeloma

Answer 10

CRAB criteria
BJP in multiple myeloma
congo red staining
skull XR

Question 11

CRAB criteria

Answer 11

high calcium
renal dysfunction
anaemia
bone disease

Question 12

what is congo red staining used for?

Answer 12

amyloid deposition in myeloma

Question 13

what is seen on skull XR in myeloma?

Answer 13

pepper pot skull/ lytic lesions (Rouleaux formation)

Question 14

management of myeloma

Answer 14

• hydration
• stop nephrotoxic drug
• chemotherapy, steroids, alkylating agents, monoclonal Ab, stem cell transplant
• Symptom control= opiates (avoid NSAIDs), local radiotherapy, bisphosphonates, vertebroplasty
• thialiomide

Question 15

what is MGUS?

Answer 15

monoclonal gammopathy of undetermined significance

precursor to myeloma

Question 16

diagnosis of MGUS

Answer 16

paraprotein <30g/l with <10% marrow plasma cells

Question 17

presentation of MGUS

Answer 17

no evidence of end organ damage

Question 18

what is AL amyloidosis?

Answer 18

mutated light chains that can precipitate in tissues to amyloid fibrils (insoluble beta-pleated sheets)

Question 19

where can the amyloid fibrils accumulate?

Answer 19

end organs causing damage

Question 20

organ damage caused by amyloid fibrils

Answer 20

nephrotic syndrome
cardiomyopathy
organomegaly, deranged LFTs
autonomic and peripheral neuropathy
malabsorption

Question 21

diagnosis of AL amyloidosis

Answer 21

biopsy with congo red stain
SAP scan
echo/ cardiac MRI
urinalysis

Question 22

management of AL amyloidosis?

Answer 22

chemotherapy

Question 23

what is Waldenstrom’s macroglobulinaemia?

Answer 23

lymphoplasmacytoid neoplasm of the IgM paraprotein

clonal disorder of cells intermediate between lymphocytes and plasma cells

Question 24

presentation of Waldenstrom’s macroglobulinaemia

Answer 24

lymphadenopathy
splenomegaly
marrow failure
hyper-viscosity syndrome
B symptoms
neuropathy

Question 25

presentation of hyper-viscosity syndrome

Answer 25

fatigue
visual disturbance
confusion
coma
bleeding
cardiac failure

triad= bleeding, visual change, neurological

Question 26

management of Waldenstrom’s macroglobulinaemia

Answer 26

chemotherapy

plasmapheresis

Question 27

management of hyperviscosity syndrome

Answer 27

remove plasma rich in IgM and replace with donor plasma