Paraproteinemias

Question 1

What ae primary paraproteinaemias?

Answer 1

Primary paraproteinaemias are caused by a build-up of monoclonal protein (either the full antibody or antibody fragments) in the serum or urine.
There may also be monoclonal plasma cells present in the:
Marrow
Soft tissue (plasmacytomas)
Circulation

Question 2

What is a pre malignant paraprotienaemia?

Answer 2

monoclonal gammopathy of unknown significance (MGUS

Patients are typically asymptomatic and have monoclonal protein on electrophoresis

Question 3

What is Waldenstrom’s macroglobulinaemia?

Answer 3

Waldenström’s macroglobulinaemia is a low-grade lymphoma (i.e. malignant) with monoclonal plasmacytoid lymphocytes that secrete monoclonal IgM found in the bone marrow and other organs.

Question 4

What are the Clincial features of Waldenstrom’s macroglobulinaemia?

Answer 4

Clinical features are caused by:
IgM deposition – hyperviscosity, polyneuropathy
organ infiltration – hepatosplenomegaly, lymphadenopathy
bone marrow infiltration – pancytopenia

Question 5

How is Waldenstrom’s macroglobulinaemia managed?

Answer 5

Watchful monitoring in asymptomatic patients
Aciclovir prophyalxis for chemotherapy
Vaccines

Question 6

What is monoclonal gammopathy of unknown significance?

Answer 6

results from an abnormal proliferation of a plasma cell clone, which leads to secretion of a monoclonal immunoglobulin. There is smaller levels of plasma less than 30 with over 10% monoclonal plasma cells and and and patients are often asymptomatic

Question 7

What does Waldenstrom’s macroglobulinaemia increase the risk of?

Answer 7

Hyperviscosity syndrome which can cause stroke, high IgM paraprotein band and filtration with lymphoplasmacytoid