6: Myeloma and other plasma cell disorders

Question 1

What are the functions of B cells?

Answer 1

Antibody production

Antigen presenting cells

Question 2

What is a terminally differentiated B cell called?

Answer 2

Plasma cell

Question 3

Every antibody has two ___ chains and two ___ chains.

Answer 3

two light chains

two heavy chains

Question 4

What shape is IgM?

Answer 4

Pentamer

Big molecule, so tends to cause hyperviscosity when produced in excess (

Question 5

Where do B cells mature?

Answer 5

Bone marrow

Question 6

By which process are a large variety of B cells produced in the bone marrow?

Answer 6

VDJ recombination

Question 7

After B cells leave the bone marrow, where do they go to be exposed to antigens?

Answer 7

Lymph node follicles

Question 8

Where in the follicle of a lymph node are B cells found?

Answer 8

Germinal centre

Question 9

In what forms are mature B cells found in the

a) bone marrow
b) circulation?

Answer 9

a) Memory B cells

b) Plasma cells

all other B cells are found in the lymph nodes, waiting to be activated

Question 10

How do plasma cells look morphologically?

Answer 10

Fried egg appearance

Nucleus off to the side, deep blue cytoplasm, white Golgi apparatus adhered to nucleus

Question 11

Blood malignancies are (polyclonal / monoclonal).

Answer 11

monoclonal

cells derived from the same dodgy parent cell

Question 12

What causes normal increases in antibody levels?

Answer 12

Infection

Inflammation

Malignancy

Question 13

What is meant by a monoclonal increase in antibodies?

Answer 13

Increase in the clone of a SINGLE B CELL

Question 14

What are monoclonal antibodies also referred to as?

Answer 14

Paraproteins

All the same because they’re all produced by the same clone of plasma cells

Question 15

Which investigation is used to separate out the different proteins found in a blood sample?

Answer 15

Serum electrophoresis

Question 16

How does normal serum electrophoresis look?

Answer 16

Variety of bands referring to variety of proteins, none of them abnormally bright

Question 17

How does serum electrophoresis look if there is a monoclonal increase in antibody levels?

Answer 17

Bands corresponding to paraproteins will be super bright

representing that there’s a ton of them being produced by a single clone of plasma cells

Question 18

Which type of chain is produced in excess by plasma cells?

Answer 18

Light chains

Question 19

Light chains are produced in excess by plasma cells.

How are these proteins excreted?

Answer 19

Via urine

Question 20

What are light chains called when they are produced in excess and excreted via the urine?

Answer 20

Bence Jones protein

A sign of myeloma (and other diseases causing increased paraproteins) found using urine electrophoresis

Question 21

What is a malignancy of plasma cells called?

Answer 21

Myeloma

Question 22

What is the benign precursor of myeloma called?

Answer 22

MGUS

Monoclonal gammopathy of unknown significance

Question 23

What are the clinical features of myeloma?

Answer 23

Hypercalcaemia symptoms: stones, bones, groans, psych moans

Polydipsia

Renal impairment (reduced eGFR, elevated urea and creatinine)

Question 24

Which types of myeloma are most common?

Answer 24

IgG myeloma

IgA myeloma

Question 25

What is the course of myeloma?

Answer 25

**relapsing**

Question 26

Why does **myeloma** cause **bony lesions?**

Answer 26

**Inflammatory cytokines cause OSTEOCLAST activation and OSTEOBLAST suppression** Causing widespread degradation (osteoporsis) and focal lesions

Question 27

Which **metabolic disturbance** occurs as a result of **bone lysis** in **myeloma?**

Answer 27

**Hypercalcaemia** bones, stones, groans and psychic moans

Question 28

What is a system commonly damaged by the **light chains** produced in myeloma?

Answer 28

**Renal** Light chains are directly toxic to the kidneys; produce **pink casts**

Question 29

**Pink casts** seen in myeloma ___ the renal tubules.

Answer 29

**block**

Question 30

How is **cast nephropathy** managed?

Answer 30

**Hydration** **Stop nephrotoxic drugs** (NSAIDs, ACEIs, ARBs, diuretics, gentamicin, metformin) **Immunosuppression** to switch off light chain production

Question 31

How is **myeloma** treated?

Answer 31

**Immunosuppression** **Chemotherapy** **Monoclonal antibodies for some types** (High dose chemo AND stem cell transplant in young, fit patients)

Question 32

How is **myeloma activity** monitored before and after treatment?

Answer 32

**Serum paraprotein levels**

Question 33

Which type of **analgesia** is given to myeloma patients for bony pain?

Answer 33

**Opiates**

Question 34

Which drugs are given to treat hypercalcaemia in myeloma patients?

Answer 34

**Bisphosphonates**

Question 35

Pathological fractures are a feature of myeloma. Where do these typically occur?

Answer 35

**Spine** (crush fractures of vertebrae)

Question 36

What is **MGUS**?

Answer 36

**Benign increase in paraprotein / plasma cell levels** Can progress to myeloma

Question 37

What is an **abnormal protein** which can accumulate in many organs to cause disease?

Answer 37

**Amyloid**

Question 38

How is **AL amyloidosis** treated?

Answer 38

**Chemo**

Question 39

How is **amyloidosis** diagnosed?

Answer 39

**Biopsy** to prove deposition **SAP scan** - nuclear scan to see deposition in other organs

Question 40

Which specific stain is used in suspected **amyloidosis**? What colour does it turn under light exposure?

Answer 40

**Congo red** **"Apple green"; birefingence**

Question 41

What is an **abnormal paraprotein, pentameric in shape**, which is produced in rare disease?

Answer 41

**IgM**

Question 42

Which disease, related to myeloma, shows an increase in **IgM paraprotein?**

Answer 42

**Waldenstrom's disease**

Question 43

What are some **symptoms** of **Waldenstrom's disease?**

Answer 43

**Hyperviscosity symptoms** - fatigue, visual problems, confusion, coma **B symptoms** - fever, weight loss, night sweats

Question 44

How is **Waldenstrom's disease** treated?

Answer 44

**Chemotherapy** - to kill off plasma cells **Plasmapharesis** - filtering of blood to remove IgM paraprotein

Question 45

How does **plasmapharesis** treat Waldenstrom's disease?

Answer 45

**Blood filtered to remove plasma which contains IgM** **Plasma replaced with donor plasma**

Question 46

What effect does **paraprotein accumulation** have on the blood?

Answer 46

**Hyperviscosity** Blood is thicker and cannot oxygenate tissues as effectively