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Haematology > Myeloma and Other Plasma Cell Dyscrasias > Flashcards

Myeloma and Other Plasma Cell Dyscrasias Flashcards

1
Q

Describe B cells

A

Derived in bone marrow from pluripotent haemopoietic stem cells
Part of the adaptive immune system

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2
Q

What are the roles of B cells?

A

Antibody production

Antigen presenting cells

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3
Q

What are immunoglobulins?

A

Antibodies produced by B cells and plasma cells

Proteins made up of 2 heavy and 2 light chains

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4
Q

What do antibodies recognise?

A

A specific antigen

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5
Q

What do B cells do regarding the periphery?

A

travel to the follicle germinal centre of the lymph node
Identify antigen and improve the fit by somatic mutation or be deleted
May return to the marrow as plasma cell or circulate as memory cell

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6
Q

Describe a plasma cell

A 
A factory cell
Pumps out antibody
Open chromatin
Plentiful blue cytoplasm (laden with protein)
Pale perinuclear area (golgi apparatus)
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7
Q

What is paraprotein?

A

Monoclonal immunoglobulin

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8
Q

What does electrophoresis detect?

A

Abnormal protein bands

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9
Q

What 2 light chains are present on antibodies?

A

Kappa

Lambda

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10
Q

What is the free light chain production by plasma cells?

A

0.5g/day

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11
Q

What are causes of paraproteinaemia?

A 
Myeloma
Amyloidosis
Lymphoma
Asymptomatic myeloma
Loads more
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12
Q

What is a myeloma?

A

A plasma cell malignancy

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13
Q

What are the direct tumour effects of myeloma?

A

Bone lesions
Increased calcium
Bone pain
Replace normal bone marrow = marrow failure

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14
Q

What other type of effects can occur from myeloma and what are they?

A

Paraprotein effects

  • renal failure
  • Immune suppression
  • Hyperviscosity
  • Amyloid
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15
Q

How is myeloma classified?

A

By the type of antibody produced

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16
Q

What are features of hypercalcaemia?

A 
Stones
Bones
Abdo groans
Psychiatric moans
Thirst
Dehydration
Renal impairement
17
Q

What effects can myeloma have on the kidney?

A 
Tubular damage by light chains
Light chain deposition - cast nephropathy
Sepsis
Hypercalcaemia and dehydration
Amyloid
Hyperuricaemia
Drugs (NSAIDs)
18
Q

How can you stop light chain production to help with cast nephropathy?

A

Steroids/chemo

19
Q

How can you treat myeloma?

A 
Corticosteroids
Alkylating agents (cyclophosphamide)
Novel agents (thalidomide)
20
Q

How do you measure the response to treatment for myeloma?

A

Paraprotein level

21
Q

How do you control the symptoms of myeloma?

A

Analgesia (not NSAIDs)
Local radiotherapy
Bisphosphonates
Vertebroplasty (inject cement into fractured bone to stabilise)

22
Q

What is MGUS?

A

Monoclonal Gammopathy of Uncertain Significance

23
Q

Describe MGUS?

A

Paraprotein <30g/l
Bone marrow plasma cells <10%
No evidence of myeloma or end organ damage

24
Q

What is AL Amyloidosis?

A

Rare
Small plasma cell clone
Mutation in the light chain > altered structure
Precipitates in tissues as an insoluble beta pleated sheet

25
Q

Describe AL Amyloidosis

A

Accumulation in tissue causes organ damage
Slowly progressive
Multisystem
Different protein to SAA amyloidosis and familial amyloidosis
Poor prognosis espeically if cardiac amyloid

26
Q

What organ damage can be caused by AL amyloid?

A 
Kidney - Nephrotic syndrome
Heart - Cardiomyopathy
Liver - Organomegaly deranged LFT's
Neuropathy - Autonomic and peripheral
GI - Malabsorption
27
Q

How do you diagnose AL amyloidosis?

A

Organ biopsy for AL amyloid deposition
Congo red stain
Rectal or fat biopsy

28
Q

How do you test for deposition in other organs?

A

SAP can
Echocardiogram
Heavy proteinuria

29
Q

How does AL amyloid look under polarised light?

A

Apple-green birefringence

30
Q

What is Waldenstrom’s Macroglobinaemia?

A

IgM paraprotein
Lymphoplasmocytoid neoplasm
Clonal disorder of cells intermediate between a lymphocyte and a plasma cell
Characteristic IgM paraprotein

31
Q

What are the effects of Waldenstrom’s Macroglobinaemia?

A 
Tumour - Lymohadenopathy 
- Splenomegaly
- Marrow failure
Paraprotein - Hyperviscosity
- Neuropathy
32
Q

Describe the IgM antibody?

A

Pentameric

33
Q

What are clinical features of Waldenstrom’s Macroglobinaemia?

A 
Hyperviscosity syndrome 
- Fatigue
- Bleeding
- Cardiac failure
Night sweats and weight loss
34
Q

How do you treat Waldenstrom’s Macroglobinaemia?

A 
Chemo
Plasmapheresis (removes protein from the circulation)

Haematology (27 decks)

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