Amyloidosis and Waldenstrom's macroglobulinaemia

Question 1

Define amyloidosis

Answer 1

Extracellular deposition of an insoluble fibrillar protein termed amyloid

Question 2

Amyloid is derived from many different precursor..

Answer 2

Proteins

Question 3

In addition to the fibrillar component, amyloid also contains a non-fibrillary proteins such as..

Answer 3

Amyloid-P component
Apolipoprotein E
heparan sulphate proteoglycans

Question 4

The accumulation of amyloid fibrils leads to..

Answer 4

Tissue/organ dysfunction

Question 5

Diagnosis of amyloidosis

Answer 5

Congo red staining: apple-green birefringence
Serum amyloid precursor (SAP) scan
Biopsy of skin, rectal mucosa, or abdominal fat

Question 6

What does the following image show?

Answer 6

Renal amyloid with congo red staining (apple-green birefringence)

Question 7

What is Waldenstrom’s macroglobulinaemia?

Answer 7

Lymphoplasmacytoid malignancy characterised by the secretion of a monoclonal IgM paraprotein

Question 8

Weight loss, lethargy
Hyperviscosity syndrome e.g. visual disturbance
Hepatosplenomegaly/lymphadenopathy
Cryoglobulinaemia e.g. Raynaud’s

Answer 8

Waldenstrom’s macroglobulinaemia

Question 9

Ix for Waldenstrom’s macroglobulinaemia

Answer 9

Monoclonal IgM paraproteinaemia
Bone marrow biopsy - diagnostic

Question 10

Biopsy findings for Waldenstrom’s macroglobulinaemia

Answer 10

Infiltration of the bone marrow with lymphoplasmacytoid lymphoma cells

Question 11

Mx of Waldenstrom’s macroglobulinaemia

Answer 11

Rituximab-based combination chemotherapy