24. Plasma Cell Proliferation

Question 1

What are plasma cell dyscrasias?

Answer 1

Neoplastic proliferation of plasma cells (ie. mature B cells) that secrete monoclonal immunoglobulins

Question 2

What is the structure of an immunoglobulin?

Answer 2

2 heavy and 2 light chains

Question 3

Classify plasma cell dyscrasias

Answer 3

Multiple myeloma
Smoldering multiple myeloma
Solitary myeloma
Monoclonal gammopathy of undetermined significance
Waldenstrom's macroglobulinaemia

Question 4

What is multiple myeloma?

Answer 4

Multifocal bone marrow disease

Malignant proliferation of plasma cells and skeletal destruction

Question 5

What immunoglobulins are usually produced in multiple myeloma?

Answer 5

IgG and IgA

Question 6

What is the name of the light chain protein excreted in urine in multiple myeloma?

Answer 6

Bence Jones protein

Question 7

What is the appearance of plasma cells?

Answer 7

Eccentric nucleus

‘Clock face’ appearance

Question 8

What are the possible causes of multiple myeloma?

Answer 8

Irradiation
Asbestos
Herpes virus 8
Genetics

Question 9

What results from the cytokines produced by myeloma cells?

Answer 9

Growth of myeloma cells
Increase osteoclast activation
Eg. IL6

Question 10

How is multiple myeloma diagnosed?

Answer 10

Radiology
Bone marrow examination
Serum and urine electrophoresis
Ig levels

Question 11

How do skeletal lesions appear in multiple myeloma?

Answer 11

‘soap bubble’
In axial skeleton
Multifocal

Question 12

What features are found in bone marrow examination in multiple myeloma?

Answer 12

Increased plasma cells
-Atypical and blasts
Russel bodies, Mott cells and Dutcher bodies
Only one type of Ig and light chain on IHC

Question 13

What is a Russel body?

Answer 13

Cell with Ig within cytoplasm

Question 14

What is a Mott cell?

Answer 14

Lots of Ig in cytoplasm

Question 15

What is a Dutcher body?

Answer 15

Ig in nucleus

Question 16

What finding is present in serum analysis of multiple myeloma?

Answer 16

Monoclonal globulin spike on electrophoresis

Question 17

What finding is present in urine electrophoresis of multiple myeloma?

Answer 17

Proteinuria with Bence Jones protein

Question 18

What are the clinical features of multiple myeloma?

Answer 18

CRAB
Hypercalcaemia
Renal failure
Anaemia
Bone lesions and bleeding

Question 19

What 3 factors cause anaemia in MM?

Answer 19

bone marrow is replaced by tumour
Reduced RBC production due to cytokines
Reduced erythropoietin production

Question 20

What is the most common cause of death for MM?

Answer 20

Sepsis

-reduction in normal Ig and leukocytes

Question 21

What is the second most common cause of death for MM?

Answer 21

Kidney failure

Question 22

What are the causes of kidney failure in MM?

Answer 22

Myeloma kidney
AL type amyloidosis
Light chain nephropathy
Increased calcium and uric acid
Pyelonephritis

Question 23

What is the difference between myeloma kidney and light chain nephropathy?

Answer 23

Myeloma kidney is due to Bence Jones protein in epithelial cells
Light chain nephropathy takes place in the glomerulus

Question 24

What is a Rouleaux formation?

Answer 24

Ig reduces the charge on RBCs so they stick together

Question 25

What is the treatment for multiple myeloma?

Answer 25

Immunomodulatory and proteasome inhibitor therapies Bisphosphonates Bone marrow transplant

Question 26

What are the symptoms of smoldering MM?

Answer 26

None

Question 27

What is solitary plasmacytoma?

Answer 27

Solitary lesions on bone or soft tissue

Question 28

What is monoclonal gammopathy of undetermined significance?

Answer 28

Early, benign stage of MM

Question 29

What age group is lymphoplasmacytic lymphoma most common in?

Answer 29

Elderly

Question 30

Why is lymphoplasmacytic lymphoma also known as?

Answer 30

Waldenstrom's macroglobulinaemia | Produces IgM which is the biggest Ig

Question 31

What are the symptoms of Waldenstrom's lymphoma?

Answer 31

``` Weakness and weight loss Lymphadenopathy Hepatosplenomegaly M spike Autoimmune haemolytic anaemia Hyperviscosity syndrome ```

Question 32

What are the symptoms of hyperviscosity syndrome?

Answer 32

Visual impairment CNS symptoms Bleeding Cryoglobulinaemia

Question 33

What causes visual impairment in hyperviscosity syndrome?

Answer 33

Distension and haemorrhage of retinal veins

Question 34

Why is there bleeding in hyperviscosity syndrome?

Answer 34

IgM binds to clotting factors and interfere with platelet function

Question 35

What is cryoglobulinaemia?

Answer 35

In cold macroglobulins stick together | Known as Raynaud's phenomenon