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Haematology Week 3 2018/19 > 6: Myeloma and other plasma cell disorders > Flashcards

6: Myeloma and other plasma cell disorders Flashcards

1
Q

What are the functions of B cells?

A

Antibody production

Antigen presenting cells

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2
Q

What is a terminally differentiated B cell called?

A

Plasma cell

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3
Q

Every antibody has two ___ chains and two ___ chains.

A

two light chains

two heavy chains

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4
Q

What shape is IgM?

A

Pentamer

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

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5
Q

Where do B cells mature?

A

Bone marrow

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6
Q

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

A

VDJ recombination

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7
Q

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

A

Lymph node follicles

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8
Q

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

A

Germinal centre

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9
Q

In what forms are mature B cells found in the

a) bone marrow
b) circulation?

A

a) Memory B cells

b) Plasma cells

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

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10
Q

How do plasma cells look morphologically?

A

Fried egg appearance

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

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11
Q

Blood malignancies are (polyclonal / monoclonal).

A

monoclonal

cells derived from the same dodgy parent cell

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12
Q

What causes normal increases in antibody levels?

A

Infection

Inflammation

Malignancy

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13
Q

What is meant by a monoclonal increase in antibodies?

A

Increase in the clone of a SINGLE B CELL

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14
Q

What are monoclonal antibodies also referred to as?

A

Paraproteins

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

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15
Q

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

A

Serum electrophoresis

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16
Q

How does normal serum electrophoresis look?

A

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

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17
Q

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

A

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

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18
Q

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

A

Light chains

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19
Q

Light chains are produced in excess by plasma cells.

How are these proteins excreted?

A

Via urine

20
Q

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

A

Bence Jones protein

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

21
Q

What is a malignancy of plasma cells called?

A

Myeloma

22
Q

What is the benign precursor of myeloma called?

A

MGUS

Monoclonal gammopathy of unknown significance

23
Q

What are the clinical features of myeloma?

A

Hypercalcaemia symptoms: stones, bones, groans, psych moans

Polydipsia

Renal impairment (reduced eGFR, elevated urea and creatinine)

24
Q

Which types of myeloma are most common?

A

IgG myeloma

IgA myeloma

25
Q

What is the course of myeloma?

A

relapsing

26
Q

Why does myeloma cause bony lesions?

A

Inflammatory cytokines cause OSTEOCLAST activation and OSTEOBLAST suppression

Causing widespread degradation (osteoporsis) and focal lesions

27
Q

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

A

Hypercalcaemia

bones, stones, groans and psychic moans

28
Q

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

A

Renal

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

29
Q

Pink casts seen in myeloma ___ the renal tubules.

A

block

30
Q

How is cast nephropathy managed?

A

Hydration

Stop nephrotoxic drugs (NSAIDs, ACEIs, ARBs, diuretics, gentamicin, metformin)

Immunosuppression to switch off light chain production

31
Q

How is myeloma treated?

A

Immunosuppression

Chemotherapy

Monoclonal antibodies for some types

(High dose chemo AND stem cell transplant in young, fit patients)

32
Q

How is myeloma activity monitored before and after treatment?

A

Serum paraprotein levels

33
Q

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

A

Opiates

34
Q

Which drugs are given to treat hypercalcaemia in myeloma patients?

A

Bisphosphonates

35
Q

Pathological fractures are a feature of myeloma.

Where do these typically occur?

A

Spine (crush fractures of vertebrae)

36
Q

What is MGUS?

A

Benign increase in paraprotein / plasma cell levels

Can progress to myeloma

37
Q

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

A

Amyloid

38
Q

How is AL amyloidosis treated?

A

Chemo

39
Q

How is amyloidosis diagnosed?

A

Biopsy to prove deposition

SAP scan - nuclear scan to see deposition in other organs

40
Q

Which specific stain is used in suspected amyloidosis?

What colour does it turn under light exposure?

A

Congo red

“Apple green”; birefingence

41
Q

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

A

IgM

42
Q

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

A

Waldenstrom’s disease

43
Q

What are some symptoms of Waldenstrom’s disease?

A

Hyperviscosity symptoms - fatigue, visual problems, confusion, coma

B symptoms - fever, weight loss, night sweats

44
Q

How is Waldenstrom’s disease treated?

A

Chemotherapy - to kill off plasma cells

Plasmapharesis - filtering of blood to remove IgM paraprotein

45
Q

How does plasmapharesis treat Waldenstrom’s disease?

A

Blood filtered to remove plasma which contains IgM

Plasma replaced with donor plasma

46
Q

What effect does paraprotein accumulation have on the blood?

A

Hyperviscosity

Blood is thicker and cannot oxygenate tissues as effectively

Haematology Week 3 2018/19 (10 decks)

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