waldenstrom's macroglobulinaemia (IgM paraprotein)

Question 1

what is it?

Answer 1

Lymphoplasmacytiod neoplasm
o Clonal disorder of cells intermediate between a lymphocyte and a plasma cell
o Charactistic IgM paraprotein

Question 2

how does it present?

Answer 2

Tumour effects
o Lymphadenopathy
o Splenomegaly
o Marrow failure

Paraprotein effects
o Hyperviscosity
o Neuropathy

Hyperviscosity syndrome
o Fatigue, visual disturbance, confusion, coma
o Bleeding
o Cardiac failure

B symptoms;
o night sweats
o weight loss

Question 3

how is it treated?

Answer 3

• Chemotherapy
• Plasmapheresis (removes paraprotein from the circulation)
• Hyperviscosity treatment –
o Remove patient plasma rich in IgM paraprotein
o Replace with donor plasma
o Viscosity reduced