Myeloma

Question 1

Definition of myeloma

Answer 1

Malignant clonal proliferation of plasma cells

Question 2

What does a monoclonal band on serum/urine electrophoresis represent? How does this differ from normal?

Answer 2

identical immunoglobulins produced by a single clone of plasma cells. Usually one plasma cell produces different immunoglobulins

Question 3

Commonest type of myeloma

Answer 3

IgG

Question 4

Why are patients with myeloma prone to infection?

Answer 4

Immunoparesis- low levels of other Ig

Question 5

What are Bence Jones proteins?

Answer 5

Free Ig light chains (kappa or lambda) appearing in the urine

Question 6

Symptoms of myeloma (5)

Answer 6

Ostelytic bone lesions causing pain, fracture
Hypercalcaemia symptoms
Bone marrow suppression symptoms
Recurrent bacterial infections
Renal impairment

Question 7

Tests in myeloma:

a) FBC
b) blood film
c) skeletal survey
d) serum and urine electrophoresis
e) bone marrow biopsy
f) urine microscopy

Answer 7

a) anaemia, may be neutropenia/thrombocytopenia if marrow infiltration
b) rouleaux formation
c) osteolytic lesions
d) monoclonal banding
e) increased plasma cells
f) casts

Question 8

Treatment of myeloma (4)

Answer 8

Corticosteroids
Alkylating agents e.g. cyclophospamide
Thalidomide
High dose chemo and autologous stem cell transplant in fit patients

Question 9

Management of bone disease (4)

Answer 9

Opiate analgesia (avoid NSAIDs)
Bisphosphonates
Vertebroplasty
Local radiotherapy

Question 10

How does myeloma cause lytic bone disease?

Answer 10

Release of IL-6 causing suppression of osteoblasts and activation of osteoclasts

Question 11

Definition of MGUS (3)

Answer 11

Paraprotein in serum

Question 12

What is the risk of MGUS?

Answer 12

Progression to myeloma

Question 13

What is amyloid?

Answer 13

Extracellular deposits of abnormal protein that are resistant to degradation

Question 14

What cause AL amyloidosis?

Answer 14

Insoluble sheets of light chain from a small mutated plasma cell clone

Question 15

How does AL amyloidosis present?

Answer 15

Slowly progressive multisystem disease

Question 16

Organ damage in AL amyloid

a) kidney
b) heart
c) liver
d) nervous
e) GI

Answer 16

a) nephrotic syndrome
b) restrictive cardiomyopathy
c) hepatomegaly and deranged LFTs
d) peripheral and autonomic neuropathy, carpal tunnel syndrome
e) malabsorption

Question 17

Tests in AL amyloid

Answer 17

isotope scan showing high uptake in affected organs

Fat/organ/rectal biopsy with Congo Red stain- “apple green birefringence” under polarised light

Question 18

Lymphoplasmacytoid neoplasm causing a characteristic IgM paraprotein

Answer 18

Waldenstrom’s macroglobulinaemia

Question 19

Tumour effects in WM (3)

Answer 19

lymphadenopathy
splenomegaly
marrow failure

Question 20

Effects of the paraprotein in WM (2)

Answer 20

Hyperviscosity

Neuropathy

Question 21

Cliical features of hyperviscosity (4)

Answer 21

Lethargy
Confusion
Visual disturbance
Cardiac failure

Question 22

Treatment of hyperviscosity

Answer 22

Plasmapharesis

Question 23

Describe the structure of antibody

Answer 23

Made of two heavy chains and two light chains; has a variable region determining antigen specificity

Question 24

How is the variable element generated?

Answer 24

From V-D-J light-chain recombination in the precursor B-cell

Question 25

What do immature B cells express on their surface and where do they go after leaving the bone marrow?

Answer 25

Express IgM and IgD, go the lymph nodes

Question 26

Responses of the B cell to activation

Answer 26

Somatic hypermutation and class switching