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Haematology > Myeloma And Plasma Cell Dyscrasias > Flashcards

Myeloma And Plasma Cell Dyscrasias Flashcards

1
Q

What are B cells derived from?

A

Pluripotent stem cells

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2
Q

What are immunoglobulins?

A

Antibodies produced by B cells and plasma cells

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3
Q

What are immunoglobulins made up of?

A

2 heavy and 2 light chains

They are a protein

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4
Q

What is the Ig region of immunoglobulin generated from?

A

V-D-J region recombination

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5
Q

What do B cells in the periphery do?

A

Travel to follicle germinal centre of lymph node
Identify antigen
May return to marrow as plasma cell or circulate as memory cell

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6
Q

What is a plasma cell?

A

A factory cell that pumps out an antibody

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7
Q

How many clones involved in polyclonal production of immunoglobulins?

A

Lots of different plasma cell clones

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8
Q

Polyclonal increase in immunoglobulins is a reaction to what?

A

Infection
Autoimmune
Malignancy
Liver disease

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9
Q

How many clones involved in monoclonal production of immunoglobulins?

A

One single B cell

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10
Q

What is a monoclonal immunoglobulin called?

A

Para protein

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11
Q

How do you detect immunoglobulins?

A

Serum electrophoresis

Serum immunofixation

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12
Q

How does free light chain production happen?

A

When Ig are synthesised in plasma cells , more light chains than heavy chains made. Free light chains are secreted into plasma with Ig.

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13
Q

Causes of paraproteinaemia ?

A 
MGUS 
Myeloma
Amyloidosis
Lymphoma 
Asymptomatic myeloma 
Solitary/ extramedullary plasmacytoma 
Chronic  lymphocytic leukaemia 
Waldenström's macroglobulinaemia
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14
Q

What is myeloma?

A

A plasma cell malignancy

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15
Q

Affects of myeloma?

A 
Bone lesions 
Increased calcium
Bone pain
Marrow failure
Renal failure
Immune suppression 
Hyperviscosity 
Amyloid
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16
Q

What is the most common myeloid?

A

IgG

17
Q

Features of hypercalcaemia?

A 
Stones
Bones
Abdo groans
Psych moans 
Renal impairment 
Dehydration 
Thirst
18
Q

Significance of renal impairment in myeloma?

A 
Tubular cell damage by light chains 
cast nephropathy 
Sepsis 
Hypercalcaemia and dehydration 
Drugs, NSAIDs
Amyloid
19
Q

How do free light chains cause renal damage?

A

Normally reabsorbed by proximal tubules
If proximal tubules overwhelmed the light chains can pass into loop of Henle
In ascending limb, tamm-horsfall protein produces and can combine with free light chains
Produces insoluble casts -> block nephron -> renal failure

20
Q

Treatment of myeloma?

A

Corticosteroids - dexamethasone , prednisolone
Alkylation agents - cyclophosphamide , lenalidomide
High dose chemo or autologous stem cell transplants

21
Q

How do you monitor response to treatment in myeloma?

A

Paraprotein level

22
Q

Myeloma symptom control?

A

Opiate analgesia - no NSAIDs
Local radiotherapy
Bisphosphonates
Vertebroplasty

23
Q

What is MGUS ?

A

Monoclonal gammopathy of uncertain significance

24
Q

Is AL amyloidosis common or rare?

A

Rare

25
Q

What is the problem in AL amyloidosis?

A

Mutation In the light chain -> altered structure which precipitates in tissues as an insoluble beta pleated sheet -> organ damage

26
Q

Prognosis in AL amyloidosis ?

A

Poor

27
Q

Organs damaged in AL amyloidosis?

A 
Kidney 
Heart
Liver
Neuropathy
GI tract
28
Q

Significant feature of organ biopsy in AL amyloidosis?

A

Congo red stain

29
Q

What paraprotein in waldenströms macroglobulinaemia?

A

IgM

30
Q

Waldenströms macroglobulinaemia is what type of neoplasia?

A

Lymphoplasmacytoid neoplasm

31
Q

Clinical features of waldenströms macroglobulinaemia?

A 
Fatigue
Visual disturbance 
Confusion
Coma
Bleeding 
Cardiac failure
Night sweats
Weight loss
32
Q

Treatment of waldenströms macroglobulinaemia?

A

Chemo

Plasmapheresis

Haematology (25 decks)

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