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Pathology (Guide) > Haematology - Multiple Myeloma + Paraproteinaemias > Flashcards

Haematology - Multiple Myeloma + Paraproteinaemias Flashcards

1
Q

What is mutliple myeloma?

A

Neoplasia of plasma cells (effector B cells + antibodies) of BM

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2
Q

What is the epidemiology mutliple myeloma?

A
  • Middle aged to elderly
  • Increased incidence in Afro-Caribbeans
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3
Q

What does multiple myeloma produce?

A
  • Monoclonal immunoglobulin (paraprotein)
  • IgG is most common
  • Paraprotein = in blood, Bence Jones Protein in urine
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4
Q

What are some clinical features of multiple myeloma?

A

CRAB:
- C: hyperCalcaemia (thirst, moans, groans, stones, bones)
- R: Renal failure (+amyloidosis + nephrotic syndrome)
- A: Anaemia (+pancytopenia)
- B: Bones (pain, osteoporosis, osteolytic lesions, fractures)

+ Hyperviscosity syndrome

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5
Q

What are some investigations for multiple myeloma?

A

Serum electrophoresis: DENSE NARROW BANDS (compared to broad band in polyclonal)
- In gamma region
- Identified as IgG, IgM, IgA, IgD, IgE
- Identified as kappa or lambda light chain

Blood film: ROULEAUX (RBC stacking)

CRAB Sx on bloods/bone profile/CT/MRI

BENCE-JONES PROTEINS (urine)

High ESR

Bone Marrow: >10% plasma cells

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6
Q

What is the staging criteria for multiple myeloma?

A

Durie-Salmon Staging

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7
Q

What is the treatment for multiple myeloma?

A
  • Supportive for CRAB Sx
  • Not curable (aim to induce remission for consideration of auto-SCT)
  1. Bortezomib +/- dexamethasone, cyclophosphamide, lenalidomide

When in remission = Auto- SCT (best for young pts as prolongs remission)

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8
Q

What is the prognosis for mutliple myeloma?

A

Poor
- Average survival: 5-7yrs (improving with new Tx)

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9
Q

What are the types of Multiple Myeloma?

A
  • Multiple Myeloma
  • Smouldering Multiple Myeloma
  • Monoclonal Gammopathy of Unknown Significance (MGUS)
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10
Q

What happens to the M-spike, bone marrow, CRAB Sx, organ damage and significance of Multiple Myeloma?

A

M spike:
- >30g/L
- Serum light free chain ratio >100

Bone Marrow:
- Any clonal plasma cell population
- Automatically diagnostic if >=60% plasma cells

CRAB:
- 1+

Organ Damage:
- Hypogammaglobulinaemia
- Occult bone disease
- Hyperviscosity
- Cytopenia

Signficance:
- Tx needed

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11
Q

What happens to the M-spike, bone marrow, CRAB Sx, organ damage and significance of Smouldering Multiple Myeloma?

A

M spike:
- >30g/L
- Serum IgA/IgG

Bone Marrow:
- >10% clonal plasma cells

CRAB:
- Nil

Organ Damage:
- Nil

Signficance:
- No Tx needed
- Higher transformation rate (most progress to MM if untreated)

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12
Q

What happens to the M-spike, bone marrow, CRAB Sx, organ damage and significance of Monoclonal Gammopathy of Unknown Significance (MGUS)?

A

M spike:
- >30g/L

Bone Marrow:
- <10% clonal plasma cells

CRAB:
- Nil

Organ Damage:
- Nil

Signficance:
- No Tx needed
- Smaller transformation rate

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13
Q

What are some general features of Waldenstrom’s Macroglobinaemia (Lymphoplasmacytoid Lymphoma)?

A
  • Rare
  • Elderly men
  • Low-grade NHL
  • LYMPHOPLASMACYTOID CELLS producing monoclonal serum IgM infiltrate in LN/BM
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14
Q

What are some clinical features of Waldenstrom’s Macroglobulinaemia (Lymphoplasmacytoid Lymphoma)?

A
  • Weight Loss
  • Fatigue
  • Hyperviscosity Syndrome
  • Lymphadenopathy
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15
Q

What are the clinical features of hyperviscosity syndrome?

A
  • Visual problems
  • Confusion
  • CCF
  • Muscle weakness
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16
Q

What is the treatment for Waldenstrom’s Macroglobulinaemia (Lymphoplasmacytoid Lymphoma)?

A
  • Plasmapharesis for Hyperviscosity
  • Rituximab/Bendamustine or Ibrutinib for Active Disease
17
Q

What is the pathophysiology of AL amyloidosis?

A
  • Due to build of mis-folded light chains
  • In presence or absence of myeloma
  • Misfolded light chains deposit in tissues + cause problems
    Results in abnormal Kappa:Lambda light chain ratio
18
Q

How is amyloidosis definitively diagnosed?

A
  • Biopsy of affected organ
  • Congo-red chain (APPLE-GREEN BIREFRINGENCE)
19
Q

What is the general presentation of amyloidosis?

A
  • Macroglossia
  • Carpal tunnel syndrome
  • Peripheral neuropathy
  • HF
  • RF

Pathology (Guide) (84 decks)

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