Paraprotein diseases

Question 1

What is Waldenstrom macroglobulinemia?

Answer 1

Low-grade lymphoma where monoclonal plasmacytoid lymphocytes in the marrow and organs secrete monoclonal IgM.

Question 2

What type of lymphoma is Waldenstrom Macroglobulinemia?

Answer 2

Low grade lymphoma

Question 3

What do monoclonal plasmacytoid lymphocytes in the marrow and organs secrete in Waldenstrom Macroglobulinemia?

Answer 3

Monoclonal IgM

Question 4

What are the clinical features of Waldenstrom macroglobulinemia caused by?

Answer 4

IgM deposition
Organ infiltration
Pancytopenia.

Question 5

What does IgM deposition cause in Waldenstrom Macroglobulinemia?

Answer 5

Hyperviscosity

Polyneuropathy

Question 6

What can hyperviscosity lead to?

Answer 6

Visual disturbance

Question 7

What can organ infiltration cause with Waldenstrom macroglobulinemia?

Answer 7

Hepatosplenomegaly

Lymphadenopathy

Question 8

Who is Waldenstrom’s macroglobulinemia seen in?

Answer 8

Older men

Question 9

What does MGUS stand for?

Answer 9

Monoclonal gammopathy of undetermined significance

Question 10

What are other terms for MGUS?

Answer 10

Benign paraproteinaemia

Monoclonal gammopathy

Question 11

What does MGUS cause?

Answer 11

Paraproteinemia

Question 12

What is MGUS often mistaken for?

Answer 12

Myeloma

Question 13

What are the main features of MGUS?

Answer 13

Asymptomatic

10-30% have demyelinating neuropathy

Question 14

What are some differing features between MGUS and myeloma?

Answer 14

• normal immune function
• normal beta-2 microglobulin levels
• lower level of paraproteinaemia than myeloma (e.g. < 30g/l IgG, or < 20g/l IgA)
• stable level of paraproteinaemia
• no clinical features of myeloma (e.g. lytic lesions on x-rays or renal disease)