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Haematology > Myeloma and Other Plasma Cell Dyscrasias > Flashcards

Myeloma and Other Plasma Cell Dyscrasias Flashcards

(44 cards)

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1
Q

What are B cells derived from?

A

Pluripotent haematopoietic stem cells in bone marrow

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2
Q

What is the role of B cells?

A

Part of adaptive immune system

Dual roles: antibody production, acting as APCs

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3
Q

What are Immunoglobulins?

A

Antibodies produced by B or plasma cells
Proteins made up of 2 heavy ((μ, α, δ, γ, ε) and 2 light chains (κ or λ)
Each Ab recognises a specific Ag

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4
Q

What is the structure of IgD, E and G?

A

Monomer

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5
Q

What is the structure of IgA?

A

Dimer

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6
Q

What is the structure of IgM?

A

Pentamer

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7
Q

What is the Ig variable element of B-cells generated from?

A

V-D-J region recombination early in development

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8
Q

Describe B cell development

A

Initial production/development in marrow
Under control/influence of microenvironment
Ig variable element generated
Self-reactive cells removed
Immature B cells with Ig on their surface exit bone marrow ready to meet target

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9
Q

From IgM Pro B cells, what is then produced?

A 
IgM and IgD Pre B cells in marrow, then:
IgM B cells, which produce
IgM plasma cells
IgA
IgE
IgG, which produce plasma cells
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10
Q

Describe B cells in the periphery

A

Travel to the follicle germinal centre of the LN
Identify the 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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11
Q

Describe plasma cells

A 
Factory cells
Pumps out Ab
Eccentric clock face nucleus on H&E
Open chromatin- synthesising mRNA
Plentiful blue cytoplasm- laden with protein
Pale perinuclear area- Golgi apparatus
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12
Q

What will polyclonal Ig’s be reactive to?

A

Infection
AI
Malignancy-reaction of host to malignant clone
Liver disease

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13
Q

What is a monoclonal Ig?

A

Paraprotein- marker of underlying clonal B-cell disorder

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14
Q

How are Ig’s detected?

A

Serum electrophoresis- separated serum proteins appear as distinct bands or zones

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15
Q

What is the most -vely charged molecule found on electrophoresis?

A

Albumin

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16
Q

What is contained in the alpha-1 band?

A

Alpha 1 antitrypsin

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17
Q

What is contained in the alpha 2 band?

A

Alpha 2 macroglobulin, caeruloplasmin, haptoglobin

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18
Q

What is contained in the beta band?

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A

Transferrin, low density lipoprotein, C3

19
Q

What is contained in the gamma band?

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A

IgG, A, M, D, E

20
Q

What is serum immunofixation used for?

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A

To classify the abnormal protein band

21
Q

What are Bence-Jones protein?

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A

Ig light chains, detected by urine electrophoresis

22
Q

What amount of free light chain is produced by normal plasma cells each day?

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A

0.5g/day

Excess can leak into the urine as BJP

23
Q

What are the causes of paraproteinaemia?

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A 
MGUS 56%
Myeloma 18%
Amyloidosis 10%
Lymphoma 5%
Asymptomatic myeloma 4%
Solitary or extramedullary plasmacytoma 3%
CLL 2%
Waldenstroms macroglobulinaemia 2%
24
Q

What are the direct tumour cell effects of clonal plasma cells in myeloma?

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A

Bone lesions
Increased calcium
Bone pain
Replacement of normal bone marrow- leading to marrow failure

25
What are the paraprotein mediated effects of clonal plasma cells in myeloma?
Renal failure Immunosuppression Hyperviscosity Amyloid
26
How is myeloma classed?
Type of Ab produced
27
What are the types of myeloma?
``` IgG 56% IgA 21% BJP myeloma- free light chain 15% Nonsecretory myeloma 3% IgD 1% Biclonal 1% IgE 0.01% ```
28
Why does myeloma cause lytic bone disease?
Increased levels of IL-6, leading to increased TGFbeta, osteoblast suppression and osteoclast activation Leads to hypercalcaemia
29
What are the symptoms and signs of hypercalcaemia?
``` Stones Bones Abdominal groans Psychiatric moans Thirst Dehydration Renal impairment ```
30
What causes damage to the kidney in myeloma?
``` Tubular cell damage by light chains Light chain deposition; cast nephropathy Sepsis Hypercalcemia and dehydration Drugs; NSAIDs Amyloid Hyperuricaemia ```
31
How is cast nephropathy managed?
Damage may be reversible with prompt treatment | Switch off light chain production with steroids/chemo
32
What is the treatment of myeloma?
Corticosteroids; dexamethasone or prednisolone Alkylating agents eg cyclophosphamide, melphalan 'Novel agents' like thalidomide, bortezomib and lenalidomide Monoclonal antibodies against plasma cells High dose chemo/autologous stem cell transplant in fit patients
33
How is myeloma response to treatment monitored?
Paraprotein level
34
How are myeloma symptoms controlled?
Opiate analgesia (avoid NSAIDs) Local radiotherapy - good for pain relief or spinal cord compression Bisphosphonates - corrects hypercalcaemia and bone pain Vertebroplasty – inject sterile cement into fractured bone to stabilise
35
What is the definition of monoclonal gammopathy of uncertain significance (MGUS)?
``` Paraprotein <30g/l Bone marrow plasma cells <10% No evidence of myeloma end organ damage; Normal calcium Normal renal function Normal Hb No lytic lesions No increase in infections ```
36
What is the risk of progression to myeloma of MGUS?
~1%
37
Describe Amyloid light chain (AL) Amyloidosis
Rare Small plasma cell clone Mutation in light chain, leads to altered structure Precipitates in tissues as an insoluble beta pleated sheet Often presents late with organ damage Accumulation in tissues causes organ damage Slowly progressive, multisystem Different protein to SAA amyloidosis/familial amyloidosis Poor prognosis esp if cardiac amyloid
38
What organ damage can AL amyloidosis cause?
``` Kidney- Nephrotic syndrome Heart- Cardiomyopathy Liver- Organomegaly deranged LFT’s Neuropathy-Autonomic, Peripheral GI tract- Malabsorption ```
39
How is AL Amyloidosis diagnosed and staged?
Organ biopsy- congo red stain, rectal or fat biopsy may be done if high clinical suspicion as less invasive Evidence of deposition in other organs- SAP scan, echo, heavy proteinuria
40
What is Waldenstrom’s Macroglobulinaemia(IgM paraprotein)?
Lymphoplasmacytoid neoplasm- clonal disorder of cells intermediate between a lymphocyte and plasma cell, characteristic IgM paraprotein
41
What are the tumour effects of Waldenstrom's?
Lymphadenopathy Splenomegaly Marrow failure
42
What are the paraprotein effects of Waldenstrom's?
Hyperviscosity | Neuropathy
43
What are the clinical features of WM?
Hyperviscosity syndrome- fatigue, visual disturbance, confusion, coma, bleeding, cardiac failure B symptoms: night sweats, weight loss
44
How is WM treated?
``` Chemo Plasmapheresis (removes paraprotein from circulation) ```

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