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- Y6 Haematology > Multiple Myeloma > Flashcards

Multiple Myeloma Flashcards

1
Q

What is multiple myeloma?

A

A haematological cancer characterised by clonal proliferation of plasma cells in the BM, typically associated with a monoclonal component in the serum and/or urine (paraprotein, usually IgG or IgA)

→ osteolytic bone disease, anaemia, and renal failure

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2
Q

What is the CRAB of multiple myeloma?

A
  • HyperCalcaemia - >2.75mmol/L
  • Renal Impairment - Cr >177 or eGFR <40
  • Anaemia - <100g/L drop of 20g/L; normochromic, normocytic
  • Bone lesions - Lytic
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3
Q

What is the epidemiology of multiple myeloma?

A
  • 2nd most common haematological cancer
  • 67yrs median age
  • M>F
  • Black > caucasian
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4
Q

What is the aetiology of multiple myeloma?

A

Unknown

  • ?Genetic inheritance
  • ?Association to ionising radiation and petroleum products
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5
Q

What are the risk factors for multiple myeloma?

A
  • MGUS
  • abnormal free light-chain ratio (kappa/lambda ratio <0.26 or >1.65 is a powerful indicator for progression to MM)
  • FH
  • radiation
  • petroleum
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6
Q

How does myeloma evolve?

A

Almost in all cases they evovle from an asymptomatic pre-malignant stage (MGUS - monoclonal gammopathy of undetermined significance0) at a rate of 1% per year.

In others there is an intermediate asymptomatic pre-malignant stage called “smouldering” myeloma. This progresses to myeloma at 10%/yr.

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7
Q

What is the protein which builds up in kidneys in multiple myeloma?

A

Bence Jones’ proteins

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8
Q

What are the symptoms of multiple myeloma?

A
  • Bone pain - osteolytic lesions in back, ribs; fractures
  • Anaemia- tiredness
  • Hyperviscosity - bleeding, headaches, visual disturbance, bruisiing
  • Infections
  • Kidney failure - (due to Bence Jones’ protein precipitating in the renal tubules)
  • Hypercalcaemia- thirst, nausea, constipation, mental change
  • Dehydration (due to proximal tubule dysfunction from light-chain precipitation)
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9
Q

What are the diagnostic features of multiple myeloma?

A

Clonal BM plasma cells ≥10%, or biopsy-proven plasmacytoma + 1 or more of:

  • Evidence of end-organ damage that can be attributed to:
    • Hypercalcaemia: serum Ca >0.25 mmol/L (>1 mg/dL) higher than upper limit of normal or >2.75 mmol/L (>11 mg/dL)
    • Renal insufficiency: Cr clearance <40 mL per minute or serum Cr >177 micromol/L (>2 mg/dL)
    • Anaemia: Hb >20 g/L below lower limit of normal, or a Hb value <100 g/L
    • Bone lesions on imaging
  • Any one or more of the following biomarkers of malignancy:
    • BM plasma cell percentage ≥60%
    • Involved:uninvolved serum free light-chain ratio ≥100
    • >1 focal lesion (each ≥5 mm) on MRI
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10
Q

What are the diagnostic features of smouldering myeloma?

A
  1. M protein (IgA or IgG) >30g/L in serum +/- BM plasma cells 10-60% +/- urinary paraprotein >500mg/24hrs AND
  2. No myeloma-defining events or amyloidosis

+ No CRAB features.

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11
Q

What are the diagnostic features of MGUS?

A
  1. M proteins <30g/L AND
  2. <10% plasma cells in BM
  3. Absence of CRAB (endo organ damage features)

(MGUS = monoclonal gammopathy of undetermined significance)

+No CRAB features.

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12
Q

What investigations would you do and what would you find?

A
  1. Immunoglobulin studies e.g. SPE, serum FLC levels, 24hr Bence Jones protein
  2. BM aspirate and biopsy - IHC for CD138
  3. FISH analysis - for high risk abnormalities
  4. Flow cytometry for immunophenotyping - diagnosis, minimal residual disease detection

Other:

  • Urinalysis - Bence Jones proteins
  • FBC - anaemia normocytic normochromic, thrombocytopenia, leukopenia
  • U&E, creatinine - impaired renal function; Cr >176
  • ESR - elevated
  • Serum calcium - high
  • Blood film - rouleaux formation with a bluish background
  • CT/PET of symptomatic areas - ?lytic lesions
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13
Q

What do these show?

  • Hb 93
  • WCC 3.14
  • Plt 87
  • MCV 101
  • Neut 1.6
  • ESR12

No abnormal white cells on differential.

A
  • Pancytopenia - all blood counts reduced
  • MCV is elevated
  • Normal/high ESR - partly due to reduced haemoglobin; but mostly due to increased protein in plasma (e.g.paraprotein in myeloma)

AML is unlikely as there are no abnormal white cells on differential.

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14
Q

When might you do a plasma exchange in myeloma?

A

Not commonly required but may have a role in patients with significantly impaired renal function at presentation.

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15
Q

Why might you get spinal compression in myeloma? What investigations would you do?

A

Vertebral damage due to myeloma–> plasmacytoma or vertebral collapse–> spinal cord compression

Investigations:

  • skeletal survey - looks for othr areas of skeletal damage which might be osteoporotic or lytic in nature
  • bone marrow aspiration - required to confirm myeloma diagnosis (excess of plasma cells)
  • MRI scan of spine - degree of bone marrow infiltration
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16
Q

What are paraproteins?

A

IgA/IgG secreted by plasma cells which do not work as intended

17
Q

What are the causes of myeloma kidney disease?

A
  • Cast nephropathy - Light chains and Tamm Horsfall glycoproteins accumulate in proximal tubule forming casts. AL amyloidosis forms by misfolded lambda chains.
  • Bence Jones proteins
  • Hypercal
  • Dehydration

Other: infection, drugs (loop diuretics, nephrotoxics)

18
Q

What are the expansile tumours of MM called?

A

Plasmacytomas

19
Q

What are the two types of abundant proteins in myeloma?

A

Bence jones = free light chains

Monoclonal IgA/G a.k.a. paraprotein/M spike

20
Q

Compare MM and Waldenstrom’s macroglobulinaemia.

A 
W = IgM producing cells
MM = IgG/IgA producing cells
21
Q
  1. If the patient has IgG/IgA MGUS what will they progress to?
  2. If the patient has IgM MGUS what will they progress to?
A
  1. MM
  2. Lymphoma
22
Q

What is the WHO diagnostic criteria for MGUS?

A
  • Serum M-protein <30g/L
  • BM clonal plasma cells <10%

AND

  • No lytic bone lesions
  • No myeloma-related organ or tissue impairment
  • No evidence of other B-cell proliferative disorder
23
Q

What criteria stratifies risk to MM progression in MGUS?

A

Mayo criteria - looks at non-IgG spike, if M-spike >15g/L and at SFLC ratio

24
Q

What are the two main criteria which define smouldering MM?

A

a). M protein >30g/L (or urinary 0.5g/day)
+/- BM plasma cells 10-60%
b) No amyloidosis and no MM defining events

25
Q

List the stages of progression of myeloma.

A
  1. MGUS - PC <10%, M <30g/L
  2. Smouldering - PC>10%, M >30g/L
  3. Symptomatic MM*
  4. Remitting relapsing MM
  5. Refractory
  6. Plasma cell leukaemia

* >10% or plasmacytoma + urine/serum paraprotein + symptoms after this stage

26
Q

What are the myeloma defining events i.e. numbers/%?

A

a) PC >60%
b) SFLC ratio >100 (invovled:uninvolved)
c) >1 focal bone lesion on MRI (>5mm)

27
Q

What are the two main emergencies in MM?

A

Cord compression and hypercalcaemia

28
Q

Which biologic may be used in MM kidney disease?

A

Bortezomib

29
Q

Name two alkylators used for MM.

A

Melphalan
Cyclophosphamide

30
Q

Name 2 steroids for MM.

A

Dexamethasone

Prednisolone

31
Q

Name 2 immunomodulatory drugs for MM.

A

-omides e.g. lenalidomide (less toxic, more potent), thalidomide

32
Q

Name 2 proteosome inhibitors for MM. Why are they useful?

A

Bortezomib
Carfilzomib
Ixazomib

Plasma cells proteosomes are crucial for removing misfolded proteins so if you remove this fucntion apoptosis of plasma cells will occur due to stress

33
Q

What is the main treatment of MM for transplant eligible and ineligible patients?

A

Transplant-eligible:

  1. Induction: PI + IMiD + Dex (x4-6) (VRD, VTD) +/- daratumumab
  2. Autologous stem cell transplant
  3. +/- consolidation (x2)(VTD, VRD)
  4. Maintenance for 2 years with low dose lenalidomide

Transplant-ineligible:

  1. Lenalidomide + dex OR
  2. Bortezomib-cyclophosphamide-dex
34
Q

Is MM curable?

A

No

35
Q

How common is MGUS?

A

most common premalignant condition

1-3% of elderly affected

increases with age

36
Q

Where are bone lesions usually found in MM? What type of imaging is used to detect these?

A

80% in PROXIMAL skeleton, spine, chest wall and pelvis

CT or FDG-PET or MRI (not XR)

37
Q

What are the features of AL amyloidosis?

A
  • nephrotic syndrome (70%) - proteinuria (not BJP), peripheral oedema
  • unexplained heart failure - biggest prognostic factor
  • sensory neuropathy
  • abnormal liver function tests
  • macroglossia
38
Q

What are the complications of AL amyloid renal disease?

A

NephrOtic syndrome → proteinuria (but not BJP)

- Y6 Haematology (22 decks)

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