Myeloma

Question 1

What are immunoglubin proteins made up of?

Answer 1

2 heavy and 2 light chains (K or lambda)

Question 2

What is a plasma cell?

Answer 2

A factory cell that pumps out antibodies
blue cytoplasm
Pale pericnuclear are which is the Golgi apparatus

Question 3

What is meant by polyclonal increase in immunoglubulins and when does this happen?

Answer 3

Produced by many different plasma cell clones

In response to infection
Autoimmune
Malignancy
Liver disease

Question 4

What is meant by monoclonal increase in immunoglobulins and when does this happen?

Answer 4

All derived from clonal expansion of a single B cell

marker of underlying clonal B cell disorder

Question 5

How are immunoglubulins detected?

Answer 5

Serum electrophoresis

Proteins move at a different rate dependent on their size and charge

Question 6

What happens to light chains that are not used in immunoglobulin synthesis?

Answer 6

They are moved into the plasma and excreted

Question 7

What is myeloma?

Answer 7

A malignancy of the plasma cells

Question 8

Direct tumour cell effects of myeloma?

Answer 8

Bone lesions
Increased calcium
Bone pain
Replace normal marrow–> marrow failure

Question 9

Paraprotein mediated effects of myeloma?

Answer 9

Renal failure
Immune suppression
Hyperviscosity
Amyloid

Question 10

How is myeloma classified?

Answer 10

By the type of immunoglobulin produced

IgG, A, E, D, nonsecretory, biclonal, BJM

Question 11

Why does myeloma cause lytic bone disease

Answer 11

myeloma cells proliferate, activate IL6 which activate osteoclasts and suppress osteoblasts

Question 12

How does light chains cause tubular damage in the kidneys?

Answer 12

Pass through glomerulus
Kidneys prevent mall proteins being absorbed into urine
If prox tubules overwhelmed, light chains pass through
Tamm Horsfall protein combines with light chains to make insoluble casts which block nephron–>Failure

Question 13

Treatment of myeloma?

Answer 13

Steroids
Alkylating agents eg cyclophosphamide
Novel agents eg thalidomide, lenolidomide
Chemo/stem cell transplant in fit patients

Question 14

Symptom control of myeloma?

Answer 14

Opiates (Avoid NSAIDs)
Local radiotherapy
Bisphosphonates
Vertebroplasty

Question 15

What is monoclonal gammopathy of uncertain significance?

Answer 15

Paraprotein < 30g/l
Bone marrow plasma cells<10%
No evidence of myeloma end organ damage

Question 16

What is AL amyloidosis?

Answer 16

Amyloid light chain amyloidosis

Mutation in light chain–> altered structure, precipitates in tissues as insoluble beta sheet

Question 17

Which organs does AL amyloidosis damage?

Answer 17

Kidney
Heart
Liver
Neuropathy
GI

Question 18

Which stain is used to detect amyloid and what are the positive results?

Answer 18

Apple green birefringence of congo red stain

Question 19

What is Waldenstrom’s macroglobulinaemia?

Answer 19

Clonal disorder of cells intermediate between a lymphocyte and a plasma cell
Characteristic IgM paraprotein

Question 20

WM clinical features?

Answer 20

Hyperviscosity syndrome
Fatigue, visual problems, confusin, coma
Bleeding
Cardiac failure

Question 21

Treatment of WM?

Answer 21

Chemotherapy

Plasmapharesis