Waldenström's macroglobulinaemia

Question 1

What is Waldenström’s macroglobulinaemia?

Answer 1

This is a lymphoplasmacytoid neoplasm

This means it is a clonal disorder of cells that are half way between lymphocytes and plasma cells

Question 2

What type of immunoglobulin is produced in WM?

Answer 2

IgM pentameric paraprotein

Question 3

What are some risk factors of WM?

Answer 3

Personal/family history of autoimmune, inflammatory or infective disorders (Especially Sjögren’s syndrome and autoimmune haemolytic anaemia)

Question 4

How will WM affect the body?

Answer 4

Direct tumour effects
Paraprotein effects

Question 5

What are some direct tumour effects of WM?

Answer 5

• Lymphadenopathy
• Splenomegaly
• Marrow failure

Question 6

What are some paraprotein effects in WM?

Answer 6

Hyperviscosity
Neuropathy

Question 7

What are some symptoms of hyper-viscosity?

Answer 7

• Fatigue
• Visual disturbance
• Confusion
• Bleeding
• Cardiac failure

Question 8

What are some general symptoms of WM?

Answer 8

Night sweats
Weight loss

Question 9

Why does WM not affect the kidneys?

Answer 9

Pentameric IgM is too large to pass into the kidneys

Question 10

What are some tests used in WM?

Answer 10

FBC
Plasma viscosity
Serum protein electrophoresis
Immunofixation
Quantification of IgM paraprotein by densitometry
Bone marrow assessment (Shows marrow infiltration by LPL)

Question 11

How is WM managed?

Answer 11

Waldenström’s macroglobulinaemia is managed using chemotherapy and plasmapheresis

Question 12

How does plasmapheresis work?

Answer 12

Plasmapheresis removes the patients blood, centrifuges it and removes the plasma, rich in IgM paraprotein and replaces it with donor plasma