Myeloma Flashcards

1
Q

What is multiple myeloma?

A

a malignant disease of the plasma cells of bone marrow, accounting for 1% of all malignant disease

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2
Q

In multiple myeloma there is clonal proliferation of what?

A

bone marrow plasma cells, usually capable of producing monoclonal immunoglobulins (paraproteins), which in most cases are IgA or IgG

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3
Q

what is paraproteinaemia and what sign is it associated with?

A

a.k.a monoclonal gammopathy

the presence of excessive amounts of paraprotein or single monoclonal gammaglobulin in the blood

excretion of light chains in the urine (Bence Jones protein) which are either kappa or lambda

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4
Q

whats the peak age of presentation?

A

60 years

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5
Q

how does bone destruction manifest clinically?

A

increased osteoclastic activity causes bone pain (back ache being the most common presenting symptom)

osteolytic lesions

pathological fractures

spinal cord compression

hypercalaemia

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6
Q

how does bone marrow infiltration manifest clinically?

A

results in anaemia, infections and bleeding

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7
Q

what causes acute kidney injury?

A

multiple causes:

deposition of light chains in the tubules
hypercalcaemia
hyperuricaemia
amyloid deposition

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8
Q

what do aggregates of paraproteins in the blood lead to?

A

they greatly increase the viscosity –> blurred vision, gangrene and bleeding

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9
Q

what are increased infections due to?

A

reduction in the normal polyclonal immunoglobulin levels (immune paresis)

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10
Q

2 out of 3 diagnostic features should be present, what are they?

A

paraproteinaemia on serum protein immunofixation or Bence Jones protein in the urine

Radiological evidence (CT, MRI) of lytic bone lesions

Increase in bone marrow plasma cells on bone marrow aspirate or trephine biopsy

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11
Q

2 other essential investigations

A

blood count: may show anaemia, thrombocytopenia and leucopenia. ESR is almost always high

serum biochemistry may show evidence of renal failure and hypercalaemia

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12
Q

median survival?

A

5 years with good supportive care and chemo with autologous stem cell transplant

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13
Q

supportive therapy

A

correction of anaemia with blood transfusion or EPO

prompt Tx of infections

Tx of bone pain with radiotherapy or high-dose dexamethasone

AKI and hypercalceamia may be corrected with adequate hydration alone

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14
Q

how is hyper viscosity treated?

A

plasma pheresis (liquid in the blood, or plasma, is separated from the cells), together with systemic therapy

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15
Q

specific Tx (involves combination chemo)

A

melphalan (an alkylating agent), prednisolone and thalidomide

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16
Q

prognostic factors

A

serum b2-microglobulin and albumin

17
Q

paraproteins

A

a protein found in the blood only as a result of cancer or other disease

18
Q

osteoclast activating factors

A

RANK ligand
MIP 1-alpha
IL-3

19
Q

adhesion of stromal cells to myeloma cells stimulates the production of what?

A

RANKL, IL-6, and also VEGF