Multiple Myeloma & Polycthemia Vera

Question 1

What is a multiple myeloma?

Answer 1

Neoplastic monoclonal proliferation of a plasma cell

Question 2

What happens in MM?

Answer 2

Typically excess production of 1 x Ig (‘monoclonal paraprotein’)
55% Ig G
20% Ig A

Question 3

Typical patient?

Answer 3

70 y/o afro carribean

Question 4

Symptoms of MM

Answer 4

Old CRAB
Old = 70+
C=hypercalaemia
R= Renal failure
A=Anemia (BM failure)
B=Bone lesions (BM failure = painful, new onset back pain in elderly)

Question 5

Why is there hypercalcemia?

Answer 5

increased osteoclast bone resorption
Bones (weak bones)
Stones (kidney stones)
Abdo moans
Psychedelic groans

Question 6

Why is there renal failure?

Answer 6

hypercalcemia = calcium oxalate renal stones

Immunoglobulin light chain kappa deposition; Bence Jones protein in Pee!!! - nephrotoxic)

Question 7

Diagnosis of MM
Tests?

Answer 7

FBC + Blood film = normocytic normochromic + high ESR, rouleaux formation (aggregation of RBCs together, abnormal)

Urine dipstick - Bence Jones protein

U&Es = Renal failure; consider XR KUB (Kidney stone)

Bone profile = hypocalemia + high ALP

Serum electrophoresis = IgM paraprotein spike; hypergammaglobulin for that specific Ig

X Ray = skull; pepper pot skull, osteolytic lesions, punched out holes

Special test = BM Biopsy >10% plasma cells

Question 8

Treatment for MM

Answer 8

Chemotherapy
Bisphosphonates (eg. alendronate) - bone protection
Dialysis
(consider BM stem cell transplant)

Question 9

What is NICE recommendation for MM?

Answer 9

Full body MRI to ID BM infiltration systemically

Question 10

What is polycthemia vera?

Answer 10

Increased blood cell level - overproduction by BM (Especially RBC)

Question 11

What is PV caused by?

Answer 11

Mutation in JAK2 gene (JAK2V617) - 95%
Activation of gene means haematopoetic stem cells divide & produce RBCs even without EPO

Question 12

Symptoms of PV?

Answer 12

Itchy (especially after a bath) - due to increased basophil & mast cells = histamine release

Burning in fingers & toes (erythromegalia)

Reddish plethoric complexion (pulpy face like cushings)

Blurry vison/headache

Hepatosplenomegaly (helps remove excel cells)

*features non specifically related to hyperviscosity = high RBC = High haematocrit = more solid mass of blood

Question 13

Diagnosis of PV?

Answer 13

FBC = High WCW, Platelets and RBC
High Hb
Genetic test = JAK2V617

Question 14

Complication of PV?

Answer 14

high risk of thromboemboli

Question 15

Treatment for PV?

Answer 15

Non curative; maintain normal blood count
1. Venesection (often used solely) - take blood out like blood test
+ aspirin
*consider chemotherapy - hydroxycarbamide aka hydroxyurea (only in high risk patients)